ABBREVIATIONS for PEDIATRIC ONCOLOGY IMAGING POLICY

AFP	Alpha-fetoprotein (tumor marker)
ALCL	Anaplastic large cell lymphoma
ALL	Acute lymphoblastic leukemia
AML	Acute myelogenous leukemia
β-hCG	Human chorionic gonadotropin beta-subunit (tumor marker)
BKL	Burkitt’s lymphoma
BWT	Bilateral Wilms tumor
CCSK	Clear cell sarcoma of the kidney
CNS	Central nervous system
COG	Children’s Oncology group
CPT®	Current procedural terminology; trademark of the American Medical Association
CSF	Cerebrospinal fluid
CT	Computed tomography
CXR	Chest x-ray
DAWT	Diffuse anaplasia Wilms tumor
ESFT	Ewing sarcoma family of tumors
FAWT	Focal anaplasia Wilms tumor
FHWT	Favorable histology Wilms tumor
HL	Hodgkin lymphoma
HSCT	Hematopoietic stem cell transplant (bone marrow or peripheral blood)
HVA	Homovanillic acid
LL	Lymphoblastic lymphoma
MIBG	Metaiodobenzylguanidine (nuclear scan using 123i or 131i)
MPNST	Malignant peripheral nerve sheath tumor
MRI	Magnetic resonance imaging
NBL	Neuroblastoma
NED	No evidence of disease
NHL	Non-hodgkin lymphoma
NPC	Nasopharyngeal carcinoma
NRSTS	Nonrhabdomyosarcomatous soft tissue sarcomas
OS	Osteosarcoma
PET	Positron emission tomography
PMBCL	Primary mediastinal b-cell lymphoma
PNET	Primitive neuroectodermal tumor
RCC	Renal cell carcinoma
RMS	Rhabdomyosarcoma
US	Ultrasound
VMA	Vannilylmandelic acid
WBC	White blood cell count
XRT	Radiation therapy

Policy:
( NOTE: For Medicare Advantage, Medicaid and FIDE-SNP, please refer to the Coverage Sections below for coverage guidance.)

PEDONC-1: General Guidelines

PEDONC-1.1: Age Considerations

PEDONC-1.2: Appropriate Clinical Evaluations

PEDONC-1.3: Modality General Considerations

PEDONC-1.4: PET Imaging in Pediatric Oncology

PEDONC-1.5: Diagnostic Radiation Exposure in Pediatric Oncology

This General Policy section provides an overview of the basic criteria for which pediatric oncology imaging may be medically necessary. Details regarding specific conditions or clinical presentations and the associated criteria for which imaging is medically necessary are described in subsequent sections.

This General Policy section provides an overview of the basic criteria for which Pediatric Oncology imaging may be medically necessary. Details regarding specific conditions or clinical presentations and the associated criteria for which imaging is medically necessary are described in subsequent sections.

PEDONC-1.1: Age Considerations

For this condition imaging is medically necessary based on the following criteria:

The majority of malignancies occurring in the pediatric population are different diagnoses than those occurring in the adult population. For those diseases which occur in both pediatric and adult populations, minor differences may exist in management between pediatric and adult medical oncologists due to member age, comorbidities, and differences in disease natural history between children and adults.

† Members who are < 18 years old at initial diagnosis should be imaged according to the Pediatric Oncology Imaging Guidelines, and members who are ≥ 18 years at initial diagnosis should be imaged according to the adult Oncology Imaging Guidelines, except where directed otherwise by a specific guideline section.

† Members who are 15 to 39 years old at initial diagnosis are defined as Adolescent and Young Adult (AYA) Oncology members. There is significantly more overlap between cancer types in this age group.

® When unique guidelines for a specific cancer type exist only in either Oncology or Pediatric Oncology, AYA members should be imaged according to the guideline section for their specific cancer type, regardless of the member’s age.

For this condition imaging is medically necessary based on the following criteria:

† In general, a recent (within 60 days) detailed history and physical examination and appropriate laboratory studies should be performed prior to considering advanced imaging, unless the member is undergoing guideline-supported scheduled off-therapy surveillance evaluation.

® Because of the relatively small number of childhood cancer treatment centers, it is common to combine off-therapy visits with imaging and other subspecialist visits to accommodate families traveling long distances for their child’s care.

® Routine imaging of brain, spine, neck, chest, abdomen, pelvis, bones, or other body areas is not indicated in the absence of localizing symptoms or abnormalities on plain radiography or ultrasound.

† For members enrolled on a COG study, imaging recommended by COG protocols should generally be approved unless the imaging is being performed solely to address a study objective and would not be indicated in usual clinical care.

Phases of Pediatric Oncology Imaging:

† Screening:

® All imaging studies requested for members at increased risk for a particular cancer in the absence of any clinical signs or symptoms.
® Screening using advanced imaging is only supported for conditions listed in PEDONC-2: Screening Imaging In Cancer Predisposition Syndromes.

® All imaging studies requested from the time cancer is first clinically suspected until the initiation of specific treatment (which may be surgical resection alone)
® Pediatric malignancies in general behave more aggressively than adult cancers, and the time from initial suspicion of cancer to specific therapy initiation can be measured in hours to days for most pediatric cancers
¡ It is recommended that children with pediatric solid tumors undergo CT evaluation of the Chest prior to general anesthesia for biopsy or resection due to the risk of post-operative atelectasis mimicking pulmonary metastasis resulting in inaccurate staging and/or delay in therapy initiation
¡ If CTs of other body areas are indicated, (Neck, Abdomen, Pelvis), they should be performed concurrently with Chest CT to avoid overlapping fields and the resulting increase in radiation exposure
¡ Metastatic CNS imaging and nuclear medicine imaging are generally deferred until after a histologic diagnosis is made, with the exception of aggressive non-Hodgkin Lymphomas

® All imaging studies completed during any type of active treatment (chemotherapy or other medications, radiation therapy, or surgery), including evaluation at the end of planned active treatment
® Unless otherwise stated in the diagnosis-specific guidelines, imaging for treatment response can be approved after every 2 cycles, which is usually ~6 weeks of therapy for solid tumors and usually ~8 to 12 weeks for CNS tumors

® All routine imaging studies requested for a member who is not receiving any active treatment, even if residual imaging abnormalities are present
® Unlike adult cancers, in most pediatric cancers surveillance does not begin until all planned multimodal therapy is completed. Pediatric cancers where surgical resection is considered curative are listed in the diagnosis-specific guideline sections
® The recommended timing for surveillance imaging studies in these guidelines refers to members who are asymptomatic or have stable chronic symptoms
® Certain tumor types do not require surveillance with advanced imaging as member outcomes following relapse are not improved by surveillance imaging. See diagnosis-specific guideline sections for details.
® PET imaging is not supported for surveillance imaging unless specifically stated in elsewhere in the diagnosis-specific guideline sections
® Members with new or changing clinical signs or symptoms suggesting recurrent disease should have symptom-appropriate imaging requests approved even when surveillance timing recommendations are not met.

® All imaging studies completed at the time a recurrence or progression of a known cancer is documented or is strongly suspected based on clinical signs or symptoms, laboratory findings, or results of basic imaging studies such as plain radiography or ultrasound
® Following documented recurrence of childhood cancer, any studies recommended for initial staging of that cancer type in the diagnosis-specific imaging guideline section should be approved
® During active treatment for recurrent pediatric cancer, conventional imaging evaluation (CT or MRI, should use the same modality for ongoing monitoring as much as possible) of previously involved areas should be approved according to the treatment response imaging in the diagnosis-specific guideline section:
¡ Imaging may be indicated more frequently than recommended by guidelines with clinical documentation that the imaging results are likely to result in a treatment change for the member, including a change from active treatment to surveillance
® Unless otherwise specified for a specific cancer type, PET is generally not indicated for routine treatment response evaluation during active treatment for recurrent pediatric cancer
¡ In rare circumstances, PET may be appropriate when results are likely to result in a treatment change for the member, including a change from active treatment to surveillance.
¡ These requests will be forwarded for Medical Director review.
® If a member with recurrent pediatric cancer completes active treatment with no evidence of disease (NED), s/he should be imaged according to the diagnosis-specific surveillance guideline sections

† Imaging performed in support of radiation therapy treatment planning should follow guidelines outlined in Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-1.5: Unlisted Procedure Codes in Oncology.

Cardiac Function Assessment in Pediatric Oncology During Active Treatment:

† Echocardiography (CPT^® 93306, CPT^® 93307, or CPT^® 93308) is preferred for evaluation of cardiac function prior to cardiotoxic chemotherapy and can be performed as often as each chemotherapy cycle at the discretion of the treating pediatric oncologist based on:

® Cumulative cardiotoxic therapy received to date
® Member’s age and gender
® Most recent echocardiogram results
® New or worsening cardiac symptoms

® Echocardiography yielded a borderline shortening fraction (< 30%) and additional left ventricular function data are necessary to make a chemotherapy decision
® Echocardiography windowing is suboptimal due to body habitus or tumor location

† Members may be severely immunocompromised during active chemotherapy treatment and any conventional imaging request to evaluate for infectious complications during this time frame should be approved immediately

† Imaging requests for infectious disease concerns for all members with absolute neutrophil count (ANC) < 500 or inconclusive findings on Chest x-ray or US at any ANC during active treatment should be approved as requested

† Additionally, members may have therapy-induced hypogammaglobulinemia which requires supplemental intravenous immune globulin (IVIG) during maintenance therapy. Members receiving supplemental IVIG should be treated similarly to members with ANC < 500 with regards to imaging for infectious disease.

† Some members are treated with very intensive chemotherapy regimens (including autologous stem cell transplantation - See Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-29: Hematopoietic Stem Cell Transplantation and spend the majority of their chemotherapy treatment phase in the hospital. Due to the high risk of invasive infections, frequent CT may be indicated to evaluate known sites of invasive fungal infection, and in general these should be approved as requested.

® Surveillance imaging of asymptomatic members to detect invasive fungal infection has not been shown to impact member outcomes. Imaging requests in these circumstances should only be approved when acute clinical decisions will be made based on the imaging.

† Transplantation of hematopoietic stem cells from bone marrow, peripheral blood, or cord blood is commonly used in the following clinical situations in pediatric hematology and oncology members:

® High risk or recurrent leukemia (allogeneic)
® Recurrent lymphoma (allogeneic or autologous)
® Hemophagocytic lymphohistiocytosis (allogeneic)
® High risk sickle cell disease (allogeneic)
® High risk neuroblastoma (autologous)
® High risk CNS tumors (autologous)
® Recurrent Ewing sarcoma family of tumors (autologous, rarely allogeneic)

For this condition imaging is medically necessary based on the following criteria:

† Plain radiography

® Chest x-ray (CXR) can provide a prompt means to evaluate primary intrathoracic tumors and continues to be the initial imaging study recommended to detect complications, such as suspected infection, in symptomatic members undergoing treatment.
® CXR continues to be the initial imaging study recommended for pulmonary surveillance for some pediatric cancers. See diagnosis-specific guideline sections for details.
® Plain radiography continues to be the initial imaging study recommended for evaluation of lesions involving the appendicular skeleton, both during and after completion of treatment. See diagnosis-specific guideline sections for details.
® Plain abdominal radiographs have been replaced by ultrasound, CT, or MRI

® Ultrasound is not widely used in pediatric oncology for staging, but is frequently used for surveillance in members who have successfully treated (primarily abdominal or pelvic) tumors with little or no residual disease. See diagnosis-specific guideline sections for details.

® CT with contrast is the imaging study of choice in pediatric members with lymphomas or solid tumors of the neck, thorax, abdomen, and/or pelvis
¡ If CT contrast use is contraindicated due to allergy or impaired renal function, either CT without contrast or MRI without and with contrast may be substituted at the discretion of the ordering physician

® MRI without and with contrast is the study of choice for CNS and musculoskeletal tumors
¡ If MRI contrast use is contraindicated due to allergy or impaired renal function, MRI without contrast may be substituted at the discretion of the ordering physician
® Due to the length of time for image acquisition and the need for stillness, anesthesia is required for almost all infants and young children (age < 7 years), as well as older children with delays in development or maturity. In this member population, MRI imaging sessions should be planned with a goal of avoiding a short-interval repeat anesthesia exposure due to insufficient information using the following considerations:
¡ MRI should always be performed without and with contrast unless there is a specific contraindication to gadolinium use, since the member already has intravenous access for anesthesia.
§ Recent evidence based literature demonstrates the potential for gadolinium deposition in various organs including the brain, after the use of MRI contrast.
§ The U.S. food and drug administration (FDA) has noted that there is currently no evidence to suggest that gadolinium retention in the brain is harmful and restricting gadolinium-based contrast agents (GBCAS) use is not warranted at this time. It has been recommended that GBCA use should be limited to circumstances in which additional information provided by the contrast agent is necessary and the necessity of repetitive MRIs with GBCAS should be assessed.
§ If requesting clinicians indicate that a non-contrast study is being requested due to concerns regarding the use of gadolinium, the exam can be approved.
¡ If multiple body areas are supported by Horizon BCBSNJ guidelines for the clinical condition being evaluated, MRI of all necessary body areas should be obtained concurrently in the same anesthesia session

® General PET imaging consideration can be found in PEDONC-1.4: PET Imaging in Pediatric Oncology.
® Bone scan is frequently used for evaluation of bone metastases during initial treatment, treatment response, and surveillance in pediatric oncology
¡ For the purposes of these guidelines, any of the following codes can be approved where “bone scan” is indicated:
§ CPT^® 78300
§ CPT^® 78305
§ CPT^® 78306
§ CPT^® 78803
§ CPT^® 78305 and CPT^® 78803
§ CPT^® 78306 and CPT^® 78803
§ If CPT^® 78300 and CPT^® 78803are requested together, only CPT^® 78803 should be approved
§ CPT^® 78315 has no specific indications for evaluation of malignant disease
® ¹²³I-metaiodobenzylguanidine (MIBG) scintigraphy is the preferred metabolic imaging for neuroblastoma and is positive in 90 to 95% of neuroblastomas, and is also used for evaluation of pheochromocytomas, paragangliomas, ganglioneuromas, and ganglioneuroblatomas
¡ For the purposes of these guidelines, any of the following codes can be approved where “MIBG” is indicated:
§ CPT^® 78800
§ CPT^® 78801
§ CPT^® 78802
§ CPT^® 78803
§ CPT^® 78804
® Octreotide and gallium scans use the same CPT codes as MIBG

For this condition imaging is medically necessary based on the following criteria:

Note: Some payors have specific restrictions on PET imaging, and those coverage policies may supersede the recommendations for PET imaging in these guidelines.

Throughout these guidelines, the term “PET” refers specifically to ¹⁸F-FDG-PET imaging and also applies to PET/CT fusion studies.

† PET imaging in pediatric Oncology should use PET/CT fusion imaging (CPT^® 78815 or CPT^® 78816) unless there is clear documentation that the treating facility does not have fusion capacity, in which case PET alone (CPT^® 78812 or CPT^® 78813) can be approved along with the appropriate CT studies. Unbundling PET/CT imaging into separate PET and diagnostic CT codes is otherwise not supported.

† The decision whether to use skull base to mid-femur (“eyes to thighs”) procedure code for PET (CPT^® 78812 or CPT^® 78815) or whole body PET (CPT^® 78813 or CPT^® 78816) is addressed in the diagnosis-specific guideline sections.

† PET imaging is not reliable for the detection of anatomic lesions smaller than 8 mm in size.

† PET imaging using isotopes other than ¹⁸F-FDG and ⁶⁸Ga-DOTATATE is considered investigational at this time.

† PET has not been shown to be diagnostically useful in all forms of childhood cancer. PET is supported for pediatric malignancies with significant published evidence regarding its diagnostic accuracy and importance in accurately directing member care decisions. See diagnosis-specific guideline sections for details.

† PET imaging is not specific to cancer, and has a high rate of false positivity. Inflammation, infection (especially granulomatous), trauma, and post-operative healing may show high levels of FDG uptake and be false-positive for malignant lesions.

† PET for rare malignancies not specifically addressed by Horizon BCBSNJ guidelines is generally not indicated, due to lack of available evidence regarding diagnostic accuracy of PET in the majority of rare cancers. Conventional imaging studies should be used for initial staging and treatment response for these diagnoses. PET can be approved if all of the following apply:

® Conventional imaging (CT, MRI, US, plain film) reveals findings that are equivocal or suspicious
® No other specific metabolic imaging (MIBG, octreotide, technetium, etc.) Is appropriate for the cancer type
® The submitted clinical information describes a specific decision regarding the member’s care that will be made based on the PET results
® These requests will be forwarded for Medical Director review

† Unless otherwise specified for a specific cancer type, once PET has been documented to be negative for a given member’s cancer or all PET-avid disease has been surgically resected, PET should not be used for continued disease monitoring or surveillance unless one of the following applies:

® Conventional imaging (CT, MRI, US, plain film) reveals findings that are inconclusive or suspicious for recurrence
¡ Residual mass that has not changed in size since the last conventional imaging does not justify PET imaging
¡ PET avidity in a residual mass at the end of planned therapy is not an indication for PET imaging during surveillance.
® Very rare circumstances where tumor markers or obvious clinical symptoms show strong evidence suggesting recurrence and PET would replace conventional imaging modalities
® The member is undergoing salvage treatment for a recurrent solid tumor with residual measurable disease on conventional imaging and confirmed repeat negative PET imaging will allow the member to transition from active treatment to surveillance.
® These requests will be forwarded for Medical Director review.

For this condition imaging is medically necessary based on the following criteria:

1. Young children are presumed to be at increased risk for malignancy from diagnostic radiation exposure, most commonly from CT and nuclear medicine imaging. They are more sensitive to radiation than adults and generally live longer after receiving radiation doses from medical procedures, resulting in a larger number of years during which to manifest a cancer.

† Because of this presumed increased risk in young children, requests to substitute MRI without and with contrast for CT with contrast to avoid radiation exposure can be approved if all of the following criteria apply:

® The member is presently a young child and the ordering physician has documented the reason for MRI, rather than CT, is to avoid radiation exposure.
® The disease-specific guidelines do not list CT as superior to MRI for the current disease and time point, meaning the MRI will provide equivalent or superior information relative to CT.
® The request is for a body area other than Chest as MRI is substantially inferior to CT for detection of small pulmonary metastases.

References

1. Krishnamurthy R, Daldrup-Link HE, Jones JY, et al. Imaging studies in the diagnosis and management of pediatric malignancies. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:185-238.
2. Allen-Rhoades W, Steuber CP. Clinical assessment and differential diagnosis of the child with suspected cancer. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:101-112.
3. Gottschalk S, Naik S, Hegde M, et al. Hematopoietic stem cell transplantation in pediatric oncology. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:419-440.
4. Freedman JL, Rheingold SR, and Fisher MJ. Oncologic emergencies. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:967-991.
5. Andrews J, Galel SA, Wong W, et al. Hematologic supportive care for children with cancer. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:992-1009.
6. Ardura MI, Koh AY. Infectious complications in pediatric cancer patients. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:1010-1057.
7. Shaikh R, Prabhu SP, Voss SD. Imaging in the evaluation and management of childhood cancer. In: Orkin SH, Fisher DE, Ginsburg D, Look AT, Lux SE, Nathan DG, eds. Nathan and Oski’s Hematology and Oncology of Infancy and Childhood. 8^th ed. Philadelphia, PA: Elsevier Saunders; 2015:2146-2254.
8. Sung L, Fisher BT, Koh AY. Infectious Disease in the pediatric cancer patient. In: Orkin SH, Fisher DE, Ginsburg D, Look AT, Lux SE, Nathan DG, eds. Nathan and Oski’s Hematology and Oncology of Infancy and Childhood. 8^th ed. Philadelphia, PA: Elsevier Saunders; 2015:2257-2266.
9. Mullen EA, Gratias E. Oncologic emergencies. In: Orkin SH, Fisher DE, Ginsburg D, Look AT, Lux SE, Nathan DG, eds. Nathan and Oski’s Hematology and Oncology of Infancy and Childhood. 8^th ed. Philadelphia, PA: Elsevier Saunders; 2015:2267-2291.
10. Weiser DA, Kaste SC, Siegel MJ, et al. Imaging in childhood cancer: A society for Pediatric Radiology and Children’s Oncology Group joint task force report. Pediatr Blood Cancer. 2013;60(8):1253-1260. doi:10.1002/pbc.24533.
11. American College of Radiology. ACR-ASER-SCBT-MR-SPR Practice Parameter for the Performance of Pediatric Computed Tomography (CT). 2014. https://www.acr.org/-/media/ACR/Files/Practice-Parameters/CT-Ped.pdf .
12. ACR-SPR Practice Parameter for the Performance and Interpretation of Pediatric Magnetic Resonance Imaging (MRI), Revised 2015 (Resolution 11). https://www.acr.org/~/media/CB384A65345F402083639E6756CE513F.pdf
13. Smith EA, Dillman JR. Current role of body MRI in pediatric oncology. Pediatr Radiol. 2016;46(6):873-880. doi:10.1007/s00247-016-3560-8.
14. The Center for Drug Evaluation and Research. Meeting of the Medical Imaging Drugs Advisory Committee, presented September 8, 2017. https://www.fda.gov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/Drugs/MedicalImagingDrugsAdvisoryCommittee/UCM574746.pdf.
15. Uslu L, Doing J, Link M, et al. Value of ¹⁸F-FDG PET and PET/CT for evaluation of pediatric malignancies. J Nucl Med. 2015;56(2):274-286. doi:10.2967/jnumed.114.146290.
16. McCarville MB. PET-CT imaging in pediatric oncology. Cancer Imaging. 2009(1);9:35-43. doi:10.1102/1470-7330.2009.0008.
17. Matthews JD, Forsythe AV, Brady Z, et al. Cancer risk in 680 000 people exposed to computed tomography scans in childhood or adolescence: data linkage study of 11 million Australians. BMJ. 2013;346:f2360. doi:10.1136/bmj.f2360.
18. Erdi YE. Limits of tumor detectability in nuclear medicine and PET. Mol Imaging Radio Nucl Ther. 2012;21(1):23-28. doi:10.4274/Mirt.138.
19. Ing C, DiMaggio C, Whitehouse A, et al. Long-term differences in language and cognitive function after childhood exposure to anesthesia. Pediatrics. 2012;130(3):e476-e485. doi:10.1542/peds.2011-3822.
20. Monteleone M, Khandji A, Cappell J, et al. Anesthesia in children: perspectives from nonsurgical pediatric specialists. J Neurosurg Anesthesiol. 2014;26(4):396-398. doi:10.1097/ANA.0000000000000124.
21. DiMaggio C, Sun LS, Li G. Early childhood exposure to anesthesia and risk of developmental and behavioral disorders in a sibling birth cohort. Anesth Analg. 2011;113(5):1143-1151. doi:10.1213/ANE.0b013e3182147f42.
22. Brody AS, Guillerman RP. Don’t let radiation scare trump patient care: 10 ways you can harm your patients by fear of radiation-induced cancer from diagnostic imaging. Thorax. 2014;69(8):782-784. doi:10.1136/thoraxjnl-2014-205499.
23. Meulepas JM, Ronckers CM, Smets AMJB, et al. Radiation exposure from pediatric CT scans and subsequent cancer risk in the Netherlands, JNCI J Natl Cancer Inst. 2019;111:256-2634. doi: 10.1093/jnci/djy104.
24. Bhatia S, Pappo AS, Acquazzino M, et al. National Comprehensive Cancer Network (NCCN) Guidelines Version 1.2020—July 11, 2019, Adolescent and Young Adult (AYA) Oncology, available at: https://www.nccn.org/professionals/physician_gls/pdf/aya.pdf, referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines™) for Adolescent and Young Adult (AYA) Oncology V1.2020 7/11/19. ^©2019 National Comprehensive Cancer Network, Inc. All rights reserved. The NCCN Guidelines™ and illustrations herein may not be reproduced in any form for any purpose without the express written permission of the NCCN. To view the most recent and complete version of the NCCN Guidelines™, go online to NCCN.org.

PEDONC-2: Screening Imaging in Cancer Predisposition Syndromes

PEDONC-2.1: Screening Imaging in Cancer Predisposition Syndromes – General Considerations

PEDONC-2.2: Li-Fraumeni Syndrome (LFS)

PEDONC-2.3: Neurofibromatosis 1 and 2 (NF1 and NF2)

PEDONC-2.4: Beckwith-Wiedemann Syndrome (BWS)

PEDONC-2.5: Denys-Drash Syndrome (DDS)

PEDONC-2.6: Wilms Tumor-Aniridia-Growth Retardation (WAGR)

PEDONC-2.7: Familial Adenomatous Polyposis (FAP) and Related Conditions

PEDONC-2.8: Multiple Endocrine Neoplasias (MEN)

PEDONC-2.9: Tuberous Sclerosis Complex (TSC)

PEDONC-2.10: Von Hippel-Lindau Syndrome (VHL)

PEDONC-2.11: Rhabdoid Tumor Predisposition Syndrome

PEDONC-2.12: Familial Retinoblastoma Syndrome

PEDONC-2.13: Hereditary Paraganglioma-Pheochromocytoma (HPP) Syndromes

PEDONC-2.14: Costello Syndrome

PEDONC-2.15: Constitutional Mismatch Repair Deficiency (CMMRD or Turcot Syndrome)

PEDONC-2.1: General Considerations

PEDONC-2.1: Screening Imaging in Cancer Predisposition Syndromes – General Considerations

For this condition imaging is medically necessary based on the following criteria:

This section is intended to give guidance for screening imaging prior to diagnosis with a specific malignancy. Once a member with a cancer predisposition syndrome has been diagnosed with a malignant disease, future imaging decisions should be guided by the appropriate disease-specific guidelines except as explicitly stated elsewhere in this section.

This section’s guidelines are limited to cancer predisposition syndromes with screening imaging considerations. Syndromes requiring only clinical or laboratory screening are not discussed here.

† In general, a recent (within 60 days) detailed history and physical examination and appropriate laboratory studies should be performed prior to considering advanced imaging, unless the member is undergoing guideline-supported scheduled screening evaluation identified in this section

† Many of these cancer predisposition syndromes also affect adults as survival continues to improve for these members. Adults with syndromes covered in this section may follow these imaging guidelines except where contradicted by specific statements in the adult imaging guidelines or payor-specific coverage policies.
Documentation of genetic or molecular confirmation of the appropriate syndrome with increased cancer risk is preferred for any member to qualify for screening imaging. There are a number of complex ethical, social, and financial issues involved in the decision to complete genetic testing in a pediatric member:

† Note: Some payors consider certain genetic tests to be investigational, and those coverage policies supersede the recommendations for genetic testing in this section.

† From the 2013 AAP Policy Statement, “Predictive genetic testing for adult-onset conditions generally should be deferred unless an intervention initiated in childhood may reduce morbidity or mortality.” Imaging surveillance is one such intervention and should not be performed without justifiable cause.

† Genetic testing should be performed in conjunction with genetic counseling for appropriate communication of risks identified by testing

† When genetic testing is not possible or not supported by health plan coverage policies, formal diagnosis after evaluation by a physician with significant training and/or experience in cancer predisposition syndromes (most commonly a geneticist or oncologist) is generally sufficient to confirm eligibility for screening imaging.

† Due to the length of time for image acquisition and the need for stillness, anesthesia is required for almost all infants and young children (age < 7 years), as well as older children with delays in development or maturity. In this member population, MRI imaging sessions should be planned with a goal of avoiding a short-interval repeat anesthesia exposure due to insufficient information using the following considerations:

® MRI should always be performed without and with contrast unless there is a specific contraindication to gadolinium use, since the member already has intravenous access for anesthesia.
¡ Recent evidence based literature demonstrates the potential for gadolinium deposition in various organs including the brain, after the use of MRI contrast.
¡ The U.S. food and drug administration (FDA) has noted that there is currently no evidence to suggest that gadolinium retention in the brain is harmful and restricting gadolinium-based contrast agents (GBCAS) use is not warranted at this time. It has been recommended that GBCA use should be limited to circumstances in which additional information provided by the contrast agent is necessary and the necessity of repetitive MRIs with GBCAS should be assessed.
¡ If requesting clinicians indicate that a non-contrast study is being requested due to concerns regarding the use of gadolinium, the exam can be approved.
® If multiple body areas are supported by Horizon BCBSNJ guidelines for the clinical condition being evaluated, MRI of all necessary body areas should be obtained concurrently in the same anesthesia session

For this condition imaging is medically necessary based on the following criteria:

Syndrome inherited in an autosomal dominant manner (50% risk to offspring) associated with germline mutations in TP53 resulted in an increased susceptibility to a variety of cancers.

† Eighty percent of individuals will have germline TP53 mutation:

® Tumor-specific TP53 mutations are much more common than germline TP53 mutations and are not associated with an increased risk for subsequent cancers
® If TP53-negative, formal diagnosis of LFS should be assigned by a physician with significant training and/or experience in LFS (most commonly a geneticist or oncologist) based on specified clinical criteria prior to beginning a screening imaging program
® TP53 mutations may be present in 50 to 80% of pediatric adrenocortical carcinoma, 10% of pediatric rhabdomyosarcoma, and 10% of pediatric osteosarcoma members

The following imaging studies should be considered appropriate in members with LFS:

Annual complete detailed physical examinations, complete blood counts, and urinalyses form the backbone of LFS cancer screening.
† Annual MRI Brain without and with contrast (CPT^® 70553) for all members

† Annual whole-body MRI (WBMRI, CPT^® 76498) for all members

† Substantial variation continues to exist in WBMRI techniques, and a specific CPT code for WBMRI has not yet been assigned. As a result, CPT^® 76498 is the only approvable code for a WBMRI study at this time.

† Abdominal (CPT^® 76700) and Pelvic (CPT^® 76856) ultrasound every 3 months from birth to age 18 (for adrenocortical carcinoma screening)

† Annual Breast MRI (CPT^® 77049) alternating every 6 months with breast ultrasound for breast cancer screening is appropriate for LFS members beginning at age 20 (See BR-6: Breast MRI Indications)

† Targeted MRI imaging without and with contrast of any body area(s) with documented signs or symptoms suggestive of possible malignancy

† When a specific malignancy is suspected, the member should be imaged according to the Horizon BCBSNJ imaging guideline specific to the suspected cancer type

† Studies ordered as part of a screening imaging program based on specific family cancer history that has been developed for an individual member in conjunction with a multidisciplinary team including at least genetics and Oncology

† Specifics of the program should be obtained and available for the Medical Director reviewing the case

PEDONC-2.3: Neurofibromatosis 1 and 2 (NF1 and NF2)

For this condition imaging is medically necessary based on the following criteria:

NF1:

Common syndrome inherited in an autosomal dominant manner (50% risk to offspring) affecting 1 in 2500 people. The diagnosis is commonly made based on established clinical criteria including café-au-lait spots, lisch nodules of the iris, axillary freckling, family history, and the presence of NF-associated tumors.

Genetic testing is encouraged for children with possible NF1 and no family history prior to assigning a diagnosis, but will not identify a mutation for all members with NF1. The majority of tumors are benign in nature, but malignant degeneration can occur. The most frequent neoplasms associated with NF1 in children are malignant peripheral nerve sheath tumor (MPNST), glioma, pheochromocytoma, and leukemia.

NF1-affected persons have increased sensitivity to ionizing radiation, so CT and nuclear medicine imaging are not appropriate screening or surveillance studies for these members. CT and/or nuclear medicine studies may be indicated for acute clinical situations and should be judged on a case-by-case basis. These requests will be forwarded for Medical Director review.

Annual ophthalmology evaluation is strongly recommended beginning at the time of diagnosis of NF1 to evaluate for optic pathway abnormalities:

† Screening MRIs of the Brain (CPT^® 70553) and Orbits (CPT^® 70543) for asymptomatic individuals are not generally recommended due to the ~60% rate of unidentified bright objects (UBOs, T2-weighted signal abnormalities) which mostly disappear by age 30

® A one-time MRI Brain (CPT^® 70553) and Orbits (CPT^® 70543) without and with contrast can be approved to clarify the diagnosis of NF1 if evaluation by a physician with significant training and/or experience in neurofibromatosis is inconclusive (most commonly a neurologist, geneticist, ophthalmologist, or oncologist)
® MRI Brain (CPT^® 70553) and Orbits (CPT^® 70543) without and with contrast can be approved for any new or worsening symptoms
® Routine follow up imaging of UBOs is not warranted in the absence of acute symptoms suggesting new or worsening intracranial disease
® Children with negative brain and orbital screening at age 15 months generally do not develop optic pathway gliomas

NF1 members are at increased risk for plexiform neurofibromas (PN) and malignant peripheral nerve sheath tumors (MPNST— a high grade sarcoma).

† Screening imaging of asymptomatic members for these tumors is not supported by evidence. PET imaging is not supported for PN surveillance in asymptomatic members at this time as the positive predictive value is only 60 to 65% even in symptomatic members.

† MRI imaging without and with contrast is appropriate for any clinical symptoms suggestive of change in a known PN in a member with NF1 (examples include pain, rapid growth, and neurologic dysfunction).

† Although PET imaging has a positive predictive value of only 61 to 63% in NF1 members with suspected transformation to MPNST, the negative predictive value is high (96 to 99%)

® PET imaging is indicated for evaluating NF1 members with clinical symptoms concerning for malignant transformation of a known PN when all of the following conditions exist:
¡ Recent MRI is inconclusive regarding transformation or progression
¡ Negative PET will result in a decision to avoid biopsy in a difficult or morbid location
® Inconclusive PET findings should lead to biopsy of the concerning lesion
¡ Repeat PET studies are not indicated due to the poor positive predictive value in this setting

† Members with NF1 and new soft tissue masses should be imaged according to Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-12: Sarcoma or PEDONC-8.3: Non-Rhabdomyosarcoma Soft Tissue Sarcomas, depending on the member’s age at the time the mass is discovered.

† Members with NF1 and new bone masses should be imaged according to PEDONC-9: Bone Tumors.

NF2:

NF2 is substantially less common than NF1. It is inherited in an autosomal dominant manner (50% risk to offspring) affecting ~1 in 25000 people. NF2 is associated with increased risk for meningiomas (50% of affected individuals), vestibular schwannomas, and spinal tumors (75% of affected individuals).

† Members with NF2 and known vestibular schwannomas should be imaged according to guidelines in Pediatric Peripheral Nerve Disorder Imaging Policy (Policy #168 in the Radiology Section); PEDPN-2.2: Neurofibromatosis 2.

† Members with NF2 and known meningioma should be imaged according to guidelines in Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-2.8: Meningiomas

† Members with NF2 and known ependymoma should be imaged according to guidelines in PEDONC-4: Ependymoma.

Recommended cancer screening imaging includes:

† Annual MRI Brain without and with contrast (CPT^® 70553) beginning at age 10 years

† MRI spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved every 3 years beginning at age 10 years for members without spinal tumors

® Annual MRI spine can be approved for all members with NF2 and a history of spinal tumors

† MRI Brain without and with contrast (CPT^® 70553) should be approved for any member with NF2 and clinical symptoms of intracranial mass or vestibular disease

† MRI spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) should be approved for any member with NF2 and:

® Clinical symptoms suggestive of new or progressive spinal or paraspinal tumors, including uncomplicated back pain or radiculopathy
® Recent diagnosis with a meningioma or vestibular schwannoma

For this condition imaging is medically necessary based on the following criteria:

Inherited syndrome characterized by macroglossia, hemihypertrophy, macrosomia, organomegaly, and neonatal hypoglycemia. Members with isolated hemihypertrophy are also imaged according to this guideline.

Caused by mutation at chromosome 11p15, affected children are predisposed to Wilms tumor, hepatoblastoma, rhabdomyosarcoma, and adrenal tumors.

Recommended cancer screening imaging includes:

† Abdominal ultrasound (CPT^® 76700) every 3 months from birth to the 8^th birthday

® Members found to have adrenal masses on screening ultrasound should receive additional imaging as follows:
¡ Purely cystic mass:
§ Continue screening ultrasound every 3 months without additional imaging
¡ Solid or mixed mass in members age 0 to 5 months:
§ If mass 0 to 3 cm in diameterMIBG imaging and either CT or MRI Abdomen (contrast as requested)
· If no evidence of malignancy based on MIBG, CT or MRI, Urine HVA/VMA, and serum ACTH, then repeat abdominal ultrasound every 6 weeks for 2 years
§ If mass > 3 cm in diameterMIBG imaging and MRI Abdomen (contrast as requested)
¡ Solid or mixed mass in members age 6 months or greater:
§ MIBG imaging prior to biopsy or resection
§ If no evidence of malignancy on biopsy or resection, resume screening abdominal ultrasound every 3 months

† Members with BWS and known hepatoblastoma should be imaged according to guidelines in PEDONC-11.2: Hepatoblastoma.

† Members with BWS and known neuroblastoma should be imaged according to guidelines in PEDONC-6: Neuroblastoma.

† Members with BWS and known adrenocortical carcinoma should be imaged according to guidelines in PEDONC-14: Pediatric Adrenocortical Carcinoma.

† Members with BWS and known pheochromocytoma should be imaged according to guidelines in Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-15: Neuroendocrine Cancers and Adrenal Tumors.

PEDONC-2.5: Denys-Drash Syndrome (DDS)

For this condition imaging is medically necessary based on the following criteria:

Characterized by pseudohermaphroditism, early renal failure, and > 90% risk of Wilms tumor development in each kidney. Associated with mutations at 11p13, risk of renal failure after detection of symptomatic Wilms tumor is 62%, so early detection may allow for renal-sparing surgical approaches.

Recommended cancer screening imaging includes:

† Abdominal ultrasound (CPT^® 76700) every 3 months from birth to the 8^th birthday

† Members with DDS and known renal tumors should be imaged according to guidelines in PEDONC-7: Pediatric Renal Tumors.

PEDONC-2.6: Wilms Tumor-Aniridia-Growth Retardation (WAGR)

For this condition imaging is medically necessary based on the following criteria:

Named for the components of the disorder, it is associated with mutations at 11p13. As the name suggests, members are predisposed to Wilms tumor, with 57% of members in one cohort developing Wilms tumor. Risk of renal failure after detection of symptomatic Wilms tumor is 38%, so early detection may allow for renal-sparing surgical approaches

Recommended cancer screening imaging includes:

† Abdominal US (CPT^® 76700) every 3 months from birth to the 8^th birthday

† Members with WAGR and known renal tumors should be imaged according to guidelines in PEDONC-7: Pediatric Renal Tumors.

PEDONC-2.7: Familial Adenomatous Polyposis (FAP) and Related Conditions

For this condition imaging is medically necessary based on the following criteria:

Inherited in an autosomal dominant manner (50% risk to offspring), it is also known as Adenomatous Polyposis Coli (APC). It is associated with the development of thousands of colonic polyps by age 20 and > 90% risk of colorectal carcinoma. Prophylactic total colectomy is recommended by age 20 for most members. FAP is also associated with hepatoblastoma, tumors of the pancreas and small bowel, medulloblastoma, and thyroid cancer.

Members with Lynch, Gardner, and Turcot syndromes should also be imaged according to these guidelines.

Recommended cancer screening imaging includes:

† Abdominal US (CPT^® 76700) every 3 months from birth to the 6^th birthday

® Annual Abdominal US for life after age 6 with family history of desmoid tumors

† Annual colonoscopy beginning at age 10

† Annual esophagogastroduodenoscopy beginning at age 10

† Annual thyroid ultrasound (CPT^® 76536) beginning at age 12

† Annual pelvic ultrasound (CPT^® 76856) beginning at age 30

† Members with FAP and known colorectal tumors should be imaged according to guidelines in Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-16: Colorectal Cancer.

† Members with FAP and known desmoid tumors should be imaged according to guidelines in PEDONC-8.3: Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS).

PEDONC-2.8: Multiple Endocrine Neoplasias (MEN)

For this condition imaging is medically necessary based on the following criteria:

Inherited in an autosomal dominant manner (50% risk to offspring)

MEN1 is characterized by parathyroid, pancreatic islet cell, and pituitary gland tumors (3 P’s), as well as carcinoid tumors in the chest and abdomen, and 28% of members will develop at least one tumor by age 15.

MEN2a is characterized by medullary thyroid carcinoma, parathyroid adenomas, and pheochromocytomas.

MEN2b is characterized by ganglioneuromas of the GI tract and skeletal abnormalities presenting in infancy.

Recommended cancer screening imaging includes:

† MEN1

® Annual MRI Brain without and with contrast (CPT^® 70553) can be approved beginning at age 5
® Annual MRI Abdomen without and with contrast (CPT^® 74183), CT Abdomen with contrast (CPT^® 74160), or ultrasound (CPT^® 76700) can be approved beginning at age 5
® Annual MRI Chest without and with contrast (CPT^® 71552) or CT Chest with contrast (CPT^® 71260) can be approved beginning at age 15
® Annual Octreotide study (CPT^® 78800, CPT^® 78801, CPT^® 78802, CPT^® 78803, or CPT^® 78804) can be approved beginning at age 5

† Members with MEN1 and known pheochromocytoma should be imaged according to guidelines in Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-15: Neuroendocrine Cancers and Adrenal Tumors

† MEN2a and MEN2b

® Annual measurement of catecholamines for pheochromocytoma screening
® MRI Abdomen without and with contrast (CPT^® 74183) can be approved every 3 years beginning at age 5
® Octreotide study (CPT^® 78800, CPT^® 78801, CPT^® 78802, CPT^® 78803, or CPT^® 78804) or Adrenal Nuclear Imaging (CPT^® 78075) can be approved for elevated catecholamines or inconclusive adrenal mass on MRI

PEDONC-2.9: Tuberous Sclerosis Complex (TSC)

For this condition imaging is medically necessary based on the following criteria:

Inherited in an autosomal dominant manner (50% risk to offspring), affecting ~1 in 6000 individuals, it is associated with benign tumors, hypopigmented skin macules (ash leaf spots), pulmonary lymphangioleiomyomatosis, developmental delay, and epilepsy.

Malignancies associated with this syndrome include:

† Subependymal giant cell astrocytomas (SEGA tumors)

® Historically, early surgery was important to reduce morbidity related to these tumors
® More recently, everolimus has been successfully used to treat these tumors without surgery, and early detection remains an important feature for success

† Cardiac rhabdomyosarcoma

† Pulmonary lymphangioleiomyomatosis

Recommended cancer screening imaging includes:

† Annual ophthalmologic and dermatologic evaluation

† Annual Brain MRI without and with contrast (CPT^® 70553) beginning at age 3 until age 25

† Annual Renal US (CPT^® 76770) beginning at age 3

® Annual MRI Abdomen without and with contrast (CPT^® 74183) can be substituted for Renal US in members with documented renal lesions

† CT Chest without contrast (CPT^® 71250) every 5 years beginning at age 18 years

® Additional CTs may be approved every 1 year for members with documented abnormalities
® CT Chest without contrast should be approved for evaluation of any new pulmonary symptoms or worsening pulmonary function testing

† Members with TSC and known renal cell carcinoma should be imaged according to PEDONC-7.4: Pediatric Renal Cell Carcinoma (RCC)

For this condition imaging is medically necessary based on the following criteria:

Inherited in an autosomal dominant manner (50% risk to offspring), it is associated with CNS hemangioblastomas, retinal angiomas, endolymphatic sac tumors (ELST), gastrointestinal stromal tumor (GIST), renal cell carcinoma (RCC), and pheochromocytomas and other neuroendocrine tumors (NETs). Pediatric members are at risk of developing hemangioblastomas and pheochromocytomas that can remain clinically occult until symptoms become severe. Historically, substantial mortality was attributable to RCC, pancreatic NET, and CNS hemangioblastoma.

Recommended cancer screening imaging includes:

† Annual ophthalmologic evaluation beginning at birth

† Annual measurement of catecholamines beginning at age 2

® Octreotide study (CPT^® 78800, CPT^® 78801, CPT^® 78802, CPT^® 78803, or CPT^® 78804) or Adrenal Nuclear imaging (CPT^® 78075) can be approved for elevated catecholamines or inconclusive adrenal mass on MRI

® If frequent ear infections are present, MRI Brain without and with contrast (CPT^® 70553) with attention to internal auditory canals can be approved

® Members with known hemangioblastoma that has not been resected can have MRI Brain every 1 year or for any new or worsening symptoms

¡ Members with known hemangioblastoma that has not been resected can have MRI Spine every 1 year or for any new or worsening symptoms

† MRI Abdomen without and with contrast (CPT^® 74183) every 2 years beginning at age 10

† Members with VHL and known CNS Hemangioblastoma should be imaged according to PEDONC-4.2: Intracranial Low Grade Gliomas (LGG)

† Members with VHL and known renal cell carcinoma should be imaged according to PEDONC-7.4: Pediatric Renal Cell Carcinoma (RCC)

† Members with VHL and known pheochromocytoma or other neuroendocrine tumors should be imaged according to guidelines in Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-15: Neuroendocrine Cancers And Adrenal Tumors

For this condition imaging is medically necessary based on the following criteria:

Inherited in an autosomal dominant manner (50% risk to offspring), it is associated with malignant rhabdoid tumors of the kidney and extrarenal locations, and atypical teratoid/rhabdoid tumors (ATRT) of the CNS. It is caused by a germline mutation in INI1 or SMARCB1, and is associated with a more variable prognosis than de novo rhabdoid tumors.

† Targeted advanced imaging should be approved for any member with this syndrome and any clinical symptoms to suggest malignancy

† Ultrasound of the head (CPT^® 76506), abdomen (CPT^® 76700), and pelvis (CPT^® 76856) monthly from birth to 12 months of age

† MRI can be approved for clarification of inconclusive findings on ultrasound, and should be used in place of ultrasound for remainder of planned screening

† MRI Brain (CPT^® 70553) and Spine (CPT^® 72156, 72157, & 72158) without and with contrast every 3 months from age 1 to 5 years

† MRI (CPT^® 74183 & 72197) or Ultrasound Abdomen and Pelvis (CPT^® 76700 & 76856) every 3 months from age 1 to 5 years

® Whole-body MRI resolution may not be sufficient to detect small rhabdoid tumors, so is not recommended in lieu of conventional MRI studies

For this condition imaging is medically necessary based on the following criteria:

This syndrome is inherited in an autosomal dominant manner (50% risk to offspring). As the name suggests, it is associated with retinoblastoma, as well as osteosarcoma, pediatric melanoma, and a significantly increased risk for radiation-related malignancies.

Regular physical and ophthalmologic evaluations under anesthesia (EUA) are the hallmark of surveillance strategies for these members, and asymptomatic screening imaging does not have a defined role at this time.

† Members with retinomas (premalignant retinal lesions) can have annual MRI Orbits (CPT^® 70543)

When advanced imaging is necessary for evaluation of inconclusive EUA findings or new symptoms, ultrasound or MRI should be used if at all possible in lieu of CT or nuclear imaging if at all possible to avoid radiation exposure in these members

PEDONC-2.13: Hereditary Paraganglioma-Pheochromocytoma (HPP) Syndromes

For this condition imaging is medically necessary based on the following criteria:

Caused by mutations in SDHx genes, this syndrome is inherited in an autosomal dominant manner (50% risk to offspring), and is associated with pheochromocytomas and paragangliomas.

Members with multiple endocrine neoplasias should not use this guideline and should be imaged according to PEDONC-2.8: Multiple Endocrine Neoplasias (MEN).

Cancer screening should begin at age 6. The following recommended imaging can be approved:

† All members with SDHx mutations:

® Annual measurement of catecholamines
® One of the following every 2 years:
¡ Whole body MRI (CPT^® 76498)
¡ MRI Neck (CPT^® 70543), Chest (CPT^® 71552), Abdomen (CPT^® 74183), Pelvis (CPT^® 72197) without and with contrast
¡ CT Neck (CPT^® 70491), Chest (CPT^® 71260), and Abdomen/Pelvis (CPT^® 74177) with contrast
¡ MRI is preferred to CT to minimize radiation exposure given these members’ lifelong need for screening

For this condition imaging is medically necessary based on the following criteria:

Caused by mutations in HRAS genes, this syndrome is inherited in an autosomal dominant manner (50% risk to offspring), and is associated with rhabdomyosarcoma and neuroblastoma in early childhood, and transitional cell cancer of the bladder in older children and adults.

Recommended Screening Imaging Includes:

† Following initial diagnosis, any or all of the following are indicated:

® Echocardiogram (CPT^® 93306)
® MRI Brain (CPT^® 70553) without and with contrast
® MRI Cervical (CPT^® 72156) and Thoracic Spine (CPT^® 72157) without and with contrast

† Echocardiogram (CPT^® 93306) as requested for members with Costello syndrome and known cardiac disease

† Members with Costello syndrome and known rhabdomyosarcoma should be imaged according to guidelines in PEDONC-8.2: Rhabdomyosarcoma (RMS)

† Members with Costello syndrome and known neuroblastoma should be imaged according to guidelines in PEDONC-6: Neuroblastoma

PEDONC-2.15: Constitutional Mismatch Repair Deficiency (CMMRD or Turcot Syndrome)

For this condition imaging is medically necessary based on the following criteria:

A highly penetrant and aggressive cancer predisposing syndrome resulting from autosomal recessive inheritance of biallelic mutations in mismatch repair genes, CMMRD syndrome leads to substantial risk for several commonly fatal childhood malignancies - high-grade CNS tumors (glioma, PNET, medulloblastoma) and hematologic malignancies (non-Hodgkin lymphoma, acute lymphoblastic leukemia). CMMRD members are also at increased risk for gastrointestinal tumors.

Recommended Screening Imaging Includes:

† MRI Brain without and with contrast (CPT^® 70553) every 6 months after CMMRD diagnosis is confirmed

† Annual whole body MRI (CPT^® 76498) beginning at age 6 years

† Annual esophagogastroduodenoscopy and colonoscopy beginning at age 4 years

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41. Ardern-Holmes S, Fisher G, North K. Neurofibromatosis Type 2: presentation, major complications, and management, with a focus on the pediatric age group. J Child Neurol. 2016;32(1):9-22. doi:10.1177/0883073816666736.
42. Shuman C, Beckwith JB, Weksberg R. Beckwith-Wiedemann syndrome. In: Pagon RA, Adam MP, Bird TD et al., eds. GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle; 2016. Version August 11, 2016. https://www.ncbi.nlm.nih.gov/pubmed/20301568.
43. Choyke PL, Siegel MJ, Craft AW, Green DM, DeBaun MR. Screening for Wilms Tumor in children with Beckwith-Wiedemann syndrome or idiopathic hemihypertrophy. Med Pediatr Oncol. 1999;32(3):196-200. doi:10.1002/(SICI)1096-911X(199903)32:3<196::AID-MPO6>3.0.CO;2-9.
44. Mussa A, Molinatto C, Baldassare G, et al. Cancer risk in Beckwith-Wiedemann syndrome: A systematic review and meta-analysis outlining a novel (epi)genotype specific histotype targeted screening protocol. J Pediatr. 2016 September;176:142-149. doi:10.1016/j.jpeds.2016.05.038.
45. MacFarland SP, Mostoufi-Moab S, Zelley K, et al. Management of adrenal masses in patients with Beckwith-Wiedemann syndrome. Pediatr Blood Cancer. 2017;64(8):e26432. doi:10.1002/pbc.26432.
46. Dome JS and Huff V. Wilms tumor predisposition. In: Pagon RA, Adam MP, Bird TD et al., eds. GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle; 2016. https://www.ncbi.nlm.nih.gov/pubmed/20301471 Accessed January 3, 2018
47. Fischbach BV, Trout KL, Lewis J, Luis CA, Sika M. WAGR Syndrome: A Clinical Review of 54 Cases. Pediatrics. 2005;116(4):984-988. doi:10.1542/peds.2004-0467.
48. Provenzale D, Gupta S, Ahnen DJ, et al. National Comprehensive Cancer Network (NCCN) Guidelines Version 3.2017—October 10, 2017, Genetic/Familial High-Risk Assessment: Colorectal, available at: https://www.nccn.org/professionals/physician_gls/pdf/genetics_colon.pdf, Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines™) for Genetic/Familial High-Risk Assessment: Colorectal V2.2019 8/8/19. ©2019 National Comprehensive Cancer Network, Inc. All rights reserved. The NCCN Guidelines™ and illustrations herein may not be reproduced in any form for any purpose without the express written permission of the NCCN. To view the most recent and complete version of the NCCN Guidelines™, go online to NCCN.org
49. Kennedy RD, Potter DD, Moir CR, Mounif E. The natural history of familial adenomatous polyposis syndrome: A 24 year review of a single center experience in screening, diagnosis, and outcomes, J Pediatr Surg 2014;49(1):82-86. doi:10.1016/j.jpedsurg.2013.09.033.
50. Jasperson KW, Patel SG, Ahnen DJ. APC-Associated Polyposis Conditions. In: Pagon RA, Adam MP, Bird TD, et al., ed. GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle; 2017. Version February 2, 2017. https://www.ncbi.nlm.nih.gov/books/NBK1345/.
51. Stoffel EM, Mangu PB, Gruber SB, et al. Hereditary colorectal cancer syndromes: American Society of Clinical Oncology clinical practice guideline endorsement of the familial risk-colorectal cancer: European Society for Medical Oncology clinical practice guidelines. J Clin Oncol. 2015;33(2):209-217. doi:10.1200/JCO.2014.58.1322.
52. Giusti F, Marini F, Brandi ML. Multiple endocrine neoplasia Type 1. In: Pagon RA, Adam MP, Ardinger HH et al, ed. GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle; 1993-2019. Version December 14, 2017. https://www.ncbi.nlm.nih.gov/books/NBK1538/.
53. Thakker RV, Newey PJ, Walls GV, et al. Clinical Practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab. 2012;97(9):2990-3011. doi:10.1210/jc.2012-1230
54. Giri D, McKay V, Weber A, Blair JC. Multiple endocrine neoplasia syndromes 1 and 2: manifestations and management in childhood and adolescence. Arch Dis Child. 2015;100(10):994-999. doi:10.1136/archdischild-2014-307028.
55. Eng C. Multiple endocrine neoplasia Type 2. In: Pagon RA, Adam MP, Ardinger HH et al, eds. GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle; 1999-2019. Version August 15, 2019. https://www.ncbi.nlm.nih.gov/books/NBK1257/.
56. Northrup H, Koenig MK, Pearson DA, Au KS. Tuberous Sclerosis Complex. In: Pagon RA, Adam MP, Ardinger HH et al, eds. GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle; Version September 3, 2015. https://www.ncbi.nlm.nih.gov/pubmed/20301399.
57. Northrup H, Koenig MK, Pearson DA, Au KS. Tuberous sclerosis complex. In: Pagon RA, Adam MP, Ardinger HH et al, eds. GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle;1993-2019. Version July 12, 2018. https://www.ncbi.nlm.nih.gov/books/NBK1220/.
58. Krueger DA, Care MM, Agricola K, Tudor C, Mays M, Franz DN. Everolimus long-term safety and efficacy in subependymal giant cell astrocytoma. Neurology. 2013;80(6):574-580. doi:10.1212/WNL.0b013e3182815428.
59. Wheless JW, Klimo P. Subependymal giant cell astrocytomas in patients with tuberous sclerosis complex: considerations for surgical or pharmacotherapeutic intervention. J Child Neurol. 2014;29(11):1562-1571. doi:10.1177/0883073813501870.
60. van Leeuwaarde RS, Ahmad S, Links TP, Giles RH. Von Hippel-Lindau Syndrome. In: Pagon RA, Adam MP, Ardinger HH et al, eds. GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle;1993-2019. Version September 6, 2018. https://www.ncbi.nlm.nih.gov/books/NBK1463/.
61. Nielsen SM, Rhodes L, Blanco I, et al. Von Hippel-lindau Disease: genetics and role of genetic counseling in a Multiple Neoplasia Syndrome. J Clin Oncol. 2016;34(18):2172-2181.
62. Rednam SP, Erez A, Druker H, et al. Von Hippel-Lindau and hereditary pheochromocytoma/paraganglioma syndromes: clinical features, genetics, and surveillance recommendations in childhood. Clin Cancer Res. 2017;23:e68-e75. doi: 10.1158/1078-0432.CCR-17-0547.
63. Kordes U, Bartelheim K, Modena P, et al. Favorable outcome of patients affected by rhabdoid tumors due to Rhabdoid Tumor Predisposition Syndrome (RTPS). Pediatr Blood Cancer. 2014;61(5):919-921. doi:10.1002/pbc.24793.
64. Sredni ST, Tomita T. Rhabdoid Tumor Predisposition Syndrome. Pediatr Dev Pathol. 2015;18(1):49-58. doi:10.2350/14-07-1531-MISC.1.
65. Nemes K, Bens S, Bourdeaut F, et al. Rhabdoid tumor predisposition syndrome. In: Pagon RA, Adam MP, Ardinger HH et al, eds. GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle; 1993-2019. Version December 7, 2017. https://www.ncbi.nlm.nih.gov/books/NBK469816/.
66. Lohmann DR, Gallie BL. Retinoblastoma. In: Pagon RA, Adam MP, Ardinger HH et al, eds. GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle; 1993-2019. Version November 21, 2018. https://www.ncbi.nlm.nih.gov/books/NBK1452/.
67. Kirmani S, Young WF. Hereditary Paraganglioma-Pheochromocytoma Syndromes. In: Pagon RA, Adam MP, Ardinger HH et al, eds. GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle; 2008-2014. Version November 6, 2014. https://www.ncbi.nlm.nih.gov/pubmed/20301715.
68. Gripp KW, Lin AE. Costello Syndrome. In: Pagon RA, Adam MP, Ardinger HH et al, eds. GeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle; 1999-2019. Version January 12, 2012. https://www.ncbi.nlm.nih.gov/books/NBK1507/.
69. Tabori U, Hansford JR, Achatz MI, et al. Clinical management and tumor surveillance recommendations of inherited mismatch repair deficiency in childhood. Clin Cancer Res. 2017;23:e32-e37. doi: 10.1158/1078-0432.CCR-17-0574.

For this condition imaging is medically necessary based on the following criteria:

The overwhelming majority of leukemias occurring in children are acute. Chronic myelogenous leukemia (CML) is rare in children, and the occurrence of chronic lymphocytic leukemia (CLL) appears to have only been reported once in pediatric members to date.

† MRI Brain without and with contrast (CPT^® 70553) can be performed in members exhibiting CNS symptoms and in members found to have high tumor burden on CSF cytology.

† There is not sufficient evidence to support the use of PET imaging for any indication in the management of acute lymphoblastic leukemia, acute myeloid leukemia, or chronic myeloid leukemia.

† Routine advanced imaging is not indicated in the evaluation and management of chronic myeloid leukemia in the absence of specific localizing clinical symptoms or clearance for hematopoietic stem cell transplantation. See Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-29: Hematopoietic Stem Cell Transplantation for imaging guidelines related to transplant.

For this condition imaging is medically necessary based on the following criteria:

† The majority of ALL members have B-precursor ALL and routine advanced imaging is not necessary.

† Members with B-precursor or T-cell lymphoblastic lymphoma without bone marrow involvement are treated similarly to leukemia members of the same cell type and should be imaged according to this guideline section.

† This section does not apply to members with mature B-cell histology (primarily Burkitt’s in children). Please refer to PEDONC-5.3: Pediatric Aggressive Mature B-Cell Non-Hodgkin Lymphomas (NHL) for guidelines for these members.

† CXR should be performed to evaluate for mediastinal mass in suspected cases or upon initial diagnosis.

® If mediastinal widening is seen on CXR, CT Chest with contrast (CPT^® 71260) is indicated immediately to evaluate for airway compression and anesthesia safety prior to attempting histologic diagnosis.
® Members with known or strongly suspected T-cell histology or other suspected lymphoblastic lymphoma involvement can have either of the following approved for initial staging purposes:
¡ CT Neck (CPT^® 70491), CT Chest (CPT^® 71260) and CT Abdomen/Pelvis (CPT^® 74177) with contrast OR
¡ PET/CT (CPT^® 78815 or 78816)

Additional imaging in lymphoblastic lymphoma:

† Follow up CT to assess response to therapy is indicated only for members with known bulky nodal disease (usually with T-cell histology) at the end of induction (~4 to 6 weeks). Members with residual masses can be evaluated with every new therapy phase (Consolidation, Interim maintenance, etc., generally every 8 to 12 weeks) until disease resolution is seen.

® PET/CT (CPT^® 78815) can be approved when residual mass ≥ 8 mm in diameter is present on recent CT imaging and there is documentation of how PET findings will affect immediate treatment decision making. These requests should be forwarded for Medical Director review.

® Members with persistent residual masses can have CT of all involved bulky nodal areas performed as part of an end of therapy evaluation

† ALL members are severely immunocompromised during the first 4 to 6 weeks of treatment (induction) and any conventional imaging request to evaluate for infectious complications during this time frame should be approved immediately.

† CT or MRI imaging requests for infectious disease concerns for ALL members with absolute neutrophil count (ANC) < 500 or inconclusive findings on chest x-ray or US at any ANC during active treatment should be approved as requested.

† Additionally, members may have therapy-induced hypogammaglobulinemia which requires supplemental intravenous immune globulin (IVIG) during maintenance therapy. Members receiving supplemental IVIG should be treated similarly to members with ANC < 500 with regards to imaging for infectious disease.

Imaging during therapy for relapsed ALL:

† Relapsed ALL members are treated with very intensive chemotherapy regimens and most members spend the majority of their chemotherapy treatment phase in the hospital. Due to the high risk of invasive infections, frequent CT or MRI imaging may be indicated to evaluate known or suspected new sites of invasive fungal or other aggressive infections, and in general these should be approved as requested.

® Surveillance imaging of asymptomatic members to detect invasive fungal infection has not been shown to impact member outcomes. Imaging requests in these circumstances should only be approved when acute clinical decisions will be made based on the imaging.

† Osteonecrosis (ON) in ALL members is a relatively common complication of ALL and its treatment, primary corticosteroids. Approximately 3% of younger children and 12 to 15% of adolescents are affected by ON at some point during therapy. The peak incidence occurs approximately one year from the time of diagnosis.

® For members with symptoms suggesting osteonecrosis, MRI without contrast or without and with contrast of the affected joint(s) can be approved.
¡ CT without contrast can be approved when MRI is contraindicated or unavailable, or for diagnosis of suspected subchondral fracture
® Screening MRI of asymptomatic members age ≤ 10 years to detect osteonecrosis has not been shown to impact member outcomes, and it is not standard to alter treatment based on imaging findings alone without symptoms
¡ A single screening MRI Bilateral Hips (CPT^® 73721 or CPT^® 73723 with modifier -50) can be approved 6 to 9 months after diagnosis for members age ≥11 years
® If osteonecrosis is detected on initial MRI, corticosteroids are often withheld during maintenance chemotherapy (but continued in earlier phases of therapy).
® In members whose symptoms have resolved and are still receiving active treatment, repeat MRI without contrast of the affected joint(s) can be approved every 2 cycles of maintenance (~every 6 months) if reintroduction of corticosteroids is being considered.
® MRI without contrast of the affected joint(s) can be approved if requested for preoperative planning in members undergoing core decompression
® See PEDONC-19.4: Osteonecrosis In Long Term Cancer Survivors for information on osteonecrosis in ALL members who have completed therapy

For this condition imaging is medically necessary based on the following criteria:

The majority of AML members do not have any bulky disease and routine advanced imaging is not necessary.

Advanced imaging may be indicated for rare members with bulky tumor masses (commonly referred to as chloromas, leukemic sarcomas, or myeloid sarcomas) noted on physical examination or other imaging such as plain film or ultrasound.

† AML members are treated with very intensive chemotherapy regimens and spend the majority of their chemotherapy treatment phase in the hospital. Due to the high risk of invasive infections, frequent CT or MRI imaging may be indicated to evaluate known sites of invasive fungal infection, and in general these should be approved as requested.

® Surveillance imaging of asymptomatic members to detect invasive fungal infection has not been shown to impact member outcomes. Imaging requests in these circumstances should only be approved when acute clinical decisions will be made based on the imaging.

1. Rabin KR, Gramatges MM, Margolin JF, et al. Acute Lymphoblastic Leukemia. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:463-497.
2. Arceci RJ, Meshinchi S. Acute Myeloid Leukemia and Myelodysplastic disorders. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:498-544.
3. Rau R, Loh ML. Myeloproliferative neoplasms of childhood. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:545-567.
4. Gutierrez A, Silverman LB. Acute Lymphoblastic Leukemia. In: Orkin SH, Fisher DE, Ginsburg D, Look AT, Lux SE, Nathan DG, eds. Nathan and Oski’s Hematology and Oncology of Infancy and Childhood. 8^th ed. Philadelphia, PA: Elsevier Saunders; 2015:1527-1554.
5. Berman JN, Look AT. Pediatric Myeloid Leukemia, Myelodysplasia, and Myeloproliferative Disease. In: Orkin SH, Fisher DE, Ginsburg D, Look AT, Lux SE, Nathan DG, eds. Nathan and Oski’s Hematology and Oncology of Infancy and Childhood. 8^th ed. Philadelphia, PA: Elsevier Saunders; 2015:1555-1613.
6. Kobos R, Shukla N, and Armstrong SA. Infant Leukemias. In: Orkin SH, Fisher DE, Ginsburg D, Look AT, Lux SE, Nathan DG, eds. Nathan and Oski’s Hematology and Oncology of Infancy and Childhood. 8^th ed. Philadelphia, PA: Elsevier Saunders; 2015:1614-1625.
7. Kaplan JA. Leukemia in children. Pediatr Rev. 2019;40:319-331. doi: 10.1542/pir.2018-0192.
8. Brown P, Inaba H, Annesley C, et al. National Comprehensive Cancer Network (NCCN) Guidelines Version 1.2020—May 30, 2019, Pediatric Acute Lymphoblastic Leukemia, available at: https://www.nccn.org/professionals/physician_gls/pdf/ped_all.pdf, Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines™) for Pediatric Acute Lymphoblastic Leukemia V1.2020 5/30/19. ©2019 National Comprehensive Cancer Network, Inc. All rights reserved. The NCCN Guidelines™ and illustrations herein may not be reproduced in any form for any purpose without the express written permission of the NCCN. To view the most recent and complete version of the NCCN Guidelines™, go online to NCCN.org.
9. Ranta S, Palomäki M, Levinsen M, et al. Role of neuroimaging in children with Acute Lymphoblastic Leukemia and central nervous system involvement at diagnosis. Pediatr Blood Cancer. 2016;64:64-70. doi:10.1002/pbc.26182/epdf.
10. Agrawal AK, Saini N, Gildengorin G et al. Is routine computed tomographic scanning justified in the first week of persistent febrile neutropenia in children with malignancies? Pediatr Blood Cancer. 2011;57(4):620-624. doi:10.1002/pbc.22974/epdf.
11. Pui C-H, Yang JJ, Hunger SP, et al. Childhood Acute Lymphoblastic Leukemia: progress through collaboration. J Clin Oncol. 2015;33(27):2938-2948. doi: 10.1200/JCO.2014.59.1636.
12. Kawedia JD, Kaste SC, Pei D, et al. Pharmacokinetic, pharmacodynamic, and pharmacogenetic determinants of osteonecrosis in children with acute lymphoblastic leukemia. Blood Journal. 2011;117(8):2340-2347. doi:10.1182/blood-2010-10-311969.
13. Marcucci G, Beltrami G, Tamburini A, et al. Bone health in childhood cancer: review of the literature and recommendations for the management of bone health in childhood cancer survivors. Ann Oncol 2019;30:908-920. doi:10.1093/annonc/mdz120.
14. Vora A. Management of osteonecrosis in children and young adults with acute lymphoblastic leukaemia. Br J Haematol. 2011;155(5):549-560. doi:10.1111/j.1365-2141.2011.08871.x.
15. Kaste SC, Pei D, Cheng C, et al. Utility of early screening magnetic resonance imaging for extensive hip osteonecrosis in pediatric patients treated with glucocorticoids. J Clin Oncol. 2015;33(6):610-615. doi:10.1200/JCO.2014.57.5480.
16. Niinimäki T, Harila-Saari A, Niinimäki R. The diagnosis and classification of osteonecrosis in patients with childhood Leukemia. Pediatr Blood Cancer. 2015;62(2):198-203. doi:10.1002/pbc.25295.
17. Murphey MD, Roberts CC, Bencardino JT, et al. Osteonecrosis of the hip. ACR Appropriateness Criteria^® 2015;1-12. https://acsearch.acr.org/docs/69420/Narrative/.
18. Karol SE, Mattano LA, Yang W, et al. Genetic risk factors for the development of osteonecrosis in children under age 10 treated for acute lymphoblastic leukemia. Blood Journal. 2016;127(5):558-564. doi:10.1182/blood-2015-10-673848.
19. Chavhan GB, Babyn PS, Nathan PC, et al. Imaging of acute and subacute toxicities of cancer therapy in children. Pediatr Radiol. 2016;46(1):9-20. doi:10.1007/s00247-015-3454-1.

For this condition imaging is medically necessary based on the following criteria:

Central nervous system tumors are the second most common form of childhood cancer, accounting for ~20% of all pediatric malignancies.

Red flag symptoms raising suspicion for CNS tumors include:

Any headache complaint from a child age ≤ 5 years

Headaches awakening from sleep

Focal findings on neurologic exam

Clumsiness (common description of gait or coordination problems in young children)

Headaches associated with morning nausea/vomiting

New onset of seizure activity with focal features

Papilledema on physical exam

† MRI is the preferred imaging modality for all pediatric CNS tumors. The primary imaging study for pediatric brain tumors is MRI Brain without and with contrast (CPT^® 70553).

® For children able to undergo MRI without sedation, MRI Brain without contrast (CPT^® 70551) can be approved if requested for initial evaluation of suspected CNS tumor.
® Younger members requiring sedation for MRI should have their initial MRI performed without and with contrast in order to avoid a second anesthesia exposure.

® Because of the significant percentage of pediatric CNS tumors occurring in the posterior fossa, CT is not a recommended study for evaluation of pediatric headache when brain tumor is clinically suspected because of its limited diagnostic accuracy in this area. MRI should be used as first line imaging in these cases.
® CT should not be used in place of MRI to avoid sedation in young children when red flag symptoms for CNS tumors are present
® CT can also be approved for evaluation of headaches related to head trauma or evaluation of skull or facial bone abnormalities

† Definitive imaging should be completed prior to considering biopsy given the high degree of morbidity associated with operating on the CNS

® Occasionally biopsy is not necessary because the imaging findings provide a definitive diagnosis. Examples include diffuse intrinsic pontine glioma and optic pathway gliomas in a member with known neurofibromatosis.

® Children may undergo very frequent imaging in the immediate perioperative period around resection or debulking of a CNS tumor due to the small anatomic spaces involved. Requests for imaging during this time period to specifically evaluate postoperative course or ventriculoperitoneal shunt functioning should, in general, be approved as requested.
® A one-time MRI Brain without and with contrast (CPT^® 70553) can be approved in the immediate preoperative period (even if another study has already been completed) to gain additional information which can be important in optimizing member outcomes, such as:
¡ Completion of additional specialized MRI sequences such as diffusion-tensor imaging, perfusion imaging, tractography, or other sequences not reported under a separate CPT^® code but not part of a routine MRI Brain series
® Repeat MRI Brain that is being requested solely for loading into operative navigation software should not be requested as a diagnostic code, but can be approved under a treatment planning code (CPT^® 76498). These requests should be forwarded for Medical Director review.

Note: Some payors have specific restrictions on MR Spectroscopy, and those coverage policies may supersede the recommendations for MRS in these guidelines.

† MRS is only supported for use in brain tumors of specified histologies where diagnostic accuracy has been established in peer-reviewed literature

® See diagnosis-specific guidelines for MRS indications

† MR spectroscopy is not indicated for routine surveillance

† Requests for MRS should be forwarded for Medical Director review

PET Brain Imaging (CPT^® 78608 and CPT^® 78609):

Note: Some payors have specific restrictions on PET Brain Metabolic imaging, and those coverage policies may supersede the recommendations for this study in these guidelines.

† PET Brain Metabolic imaging (CPT^® 78608) is only supported for use in brain tumors of specified histologies where diagnostic accuracy has been established in peer-reviewed literature

® See diagnosis-specific guidelines for PET indications

† PET Brain Perfusion imaging (CPT^® 78609) is not indicated in the evaluation or management of primary CNS tumors

† Fusion PET/CT studies (CPT^® 78814, CPT^® 78815, or CPT^® 78816) are not indicated in the evaluation or management of primary CNS tumors

† PET Brain Metabolic is not indicated for routine surveillance

† Requests for PET Brain Metabolic should be forwarded for Medical Director review

For this condition imaging is medically necessary based on the following criteria:

Account for 40 to 60% of pediatric CNS tumors. These tumors are defined as having a WHO histologic grade of I or II (out of IV), can occur anywhere in the CNS, and includes the following tumors:

® Pilocytic Astrocytoma
® Fibrillary (or Diffuse) Astrocytoma
® Optic Pathway Gliomas
® Pilomyxoid Astrocytoma
® Oligodendroglioma
® Oligoastrocytoma
® Oligodendrocytoma
® Subependymal Giant Cell Astrocytoma (SEGA)
® Ganglioglioma
® Gangliocytoma
® Dysembryoplastic Infantile Astrocytoma (DIA)
® Dysembryoplastic Infantile Ganglioglioma (DIG)
® Dysembryoplastic Neuroepithelial Tumor (DNT)
® Tectal Plate Gliomas
® Cervicomedullary Gliomas
® Pleomorphic Xanthoastrocytoma (PXA)
® Any other glial tumor with a WHO grade of I or II

® To determine need for biopsy when transformation to high grade glioma is suspected based on clinical symptoms or recent MRI findings
® To evaluate a brain lesion of indeterminate nature when the PET findings will be used to determine whether biopsy/resection can be safely postponed

® To distinguish low grade from high grade gliomas
® To evaluate a brain lesion of indeterminate nature when the MRS findings will be used to determine whether biopsy/resection can be safely postponed
® To distinguish radiation-induced tumor necrosis from progressive disease within 18 months of completing radiotherapy.

Low Grade Gliomas Initial Staging:

† MRI Brain without and with contrast (CPT^® 70553) is indicated for all LGG

† MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved for all LGG members if requested, and spinal imaging is particularly recommended for members with:

® Multicentric tumors
® Intracranial leptomeningeal disease
® Clinical signs or symptoms suggesting spinal cord involvement
® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain.

Low Grade Gliomas Treatment Response:

† Children who have resection of the tumor can have a single MRI Brain without and with contrast (CPT^® 70553) approved following resection to establish baseline imaging and those with a complete resection should then be imaged according to surveillance guidelines

† Children with neurofibromatosis and small optic pathway gliomas may be observed without specific treatment and should be imaged according to surveillance guidelines for LGG.

† Members age > 10 years with incompletely resected tumors usually receive adjuvant radiation therapy and can have a single MRI Brain without and with contrast (CPT^® 70553) approved at completion of radiotherapy and should then be imaged according to surveillance guidelines

† Members age ≤ 10 years with incompletely resected tumors are commonly treated with chemotherapy and can have MRI Brain without and with contrast (CPT^® 70553) approved every 2 cycles during active treatment and at the end of planned chemotherapy

† Spinal imaging is not indicated during treatment response for members without evidence of spinal cord involvement at initial diagnosis

† Spinal imaging is appropriate every 2 cycles during induction chemotherapy for members with measurable spinal cord disease on MRI

Low Grade Gliomas Surveillance:

† MRI Brain without and with contrast (CPT^® 70553) can be approved after completion of therapy every 3 months for 2 years, then every 6 months for 3 years, then annually thereafter.

® MRI Orbits without and with contrast (CPT^® 70543) can be approved for members with optic pathway glioma and either a history of intra-orbital involvement or a history of NF1

† For members with cord involvement at diagnosis, MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved after completion of therapy every 3 months for 2 years, then every 6 months for 3 years, then annually thereafter.

® MRI Spine with contrast only can be approved (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) if being performed immediately following a contrast-enhanced MRI Brain

For this condition imaging is medically necessary based on the following criteria:

Rare in children compared with the adult population, but represent 10 to 20% of pediatric CNS tumors. Prognosis is very poor, and survival significantly beyond 3 years from diagnosis is rare, even with complete surgical resection at initial diagnosis.

These tumors are defined as having a WHO histologic grade of III or IV (out of IV) can occur anywhere in the CNS (though the majority occur in the brain), and includes the following tumors:

® Anaplastic astrocytoma
® Glioblastoma multiforme
® Diffuse intrinsic pontine glioma (DIPG, or “Brainstem glioma”)
® Gliomatosis cerebri
® Gliosarcoma
® Anaplastic oligodendroglioma
® Anaplastic ganglioglioma
® Anaplastic mixed glioma
® Anaplastic mixed ganglioneuronal tumors
® Any other glial tumor with a WHO grade of III or IV

® To distinguish radiation-induced tumor necrosis from progressive disease within 18 months of completing radiotherapy
® To evaluate inconclusive MRI findings when the PET findings will be used to determine need for biopsy or change in therapy, including a change from active therapy to surveillance
® To evaluate a brain lesion of indeterminate nature when the PET findings will be used to determine whether biopsy/resection can be safely postponed
® PET Brain is not indicated in gliomas occurring in the brain stem due to poor uptake and lack of impact on member outcomes

® To distinguish low grade from high grade gliomas
® To evaluate a brain lesion of indeterminate nature when the MRS findings will be used to determine whether biopsy/resection can be safely postponed
® To distinguish radiation-induced tumor necrosis from progressive disease within 18 months of completing radiotherapy.

High Grade Gliomas Initial Staging:

† MRI Brain without and with contrast (CPT^® 70553) is indicated for all HGG

† MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved for all HGG members if requested, and spinal imaging is particularly recommended for members with:

® Multicentric tumors
® Intracranial leptomeningeal disease
® Clinical signs or symptoms suggesting spinal cord involvement
® MRI spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

† Members who have resection of the tumor can have a single MRI Brain without and with contrast (CPT^® 70553) approved following resection to establish baseline imaging and those with a complete resection should then be imaged according to surveillance guidelines

† If receiving adjuvant radiotherapy after a completely resected tumor, an additional MRI Brain without and with contrast (CPT^® 70553) can be approved at the end of radiotherapy

† Members with incompletely resected tumors are commonly treated with chemotherapy and can have MRI Brain without and with contrast (CPT^® 70553) approved every 2 cycles during active treatment and at the end of planned chemotherapy

† Spinal imaging is not indicated during treatment response for members without evidence of spinal cord involvement at initial diagnosis

† Spinal imaging is appropriate every 2 cycles during induction chemotherapy for members with measurable spinal cord disease on MRI

High Grade Gliomas Surveillance:

† MRI Brain without and with contrast (CPT^® 70553) can be approved after completion of therapy every 3 months for 3 years, then every 6 months thereafter

† MRI Spine is not indicated during surveillance in members without prior history of spinal involvement except to evaluate symptoms suspicious for spinal cord recurrence

† For members with cord involvement at diagnosis, MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved after completion of therapy every 3 months for 3 years, then every 6 months thereafter

® MRI Spine can be performed with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) if being performed immediately following a contrast-enhanced MRI Brain

For this condition imaging is medically necessary based on the following criteria:

Account for 15 to 25% of pediatric CNS tumors, prognosis is generally favorable. Leptomeningeal spread is common and can occur after initial diagnosis.

Includes the following tumors:

† Medulloblastoma and Pineoblastoma † sPNET ® Medulloepithelioma ® Cerebral or cerebellar neuroblastoma ® Cerebral or cerebellar ganglioneuroblastoma ® Ependymoblastoma

Risk assessment is important in determining optimal treatment High risk features include the following:

† Spinal metastasis (including cytology positive only) † Multifocal intracranial tumors † Anaplastic histology † All sPNET and pineoblastomas † > 1.5 cm2 residual tumor area on postoperative MRI and age < 3 years

Members without any high risk features are considered “average risk”

† PET Brain Metabolic Imaging (CPT^® 78608) can be approved in the following circumstances:

® To distinguish radiation-induced tumor necrosis from progressive disease within 18 months of completing radiotherapy
® To evaluate inconclusive MRI findings when the PET findings will be used to determine need for biopsy or change in therapy, including a change from active therapy to surveillance
® To evaluate a Brain lesion of indeterminate nature when the PET findings will be used to determine whether biopsy/resection can be safely postponed

® To evaluate a brain lesion of indeterminate nature when the MRS findings will be used to determine whether biopsy/resection can be safely postponed

† Preoperative MRI Brain without and with contrast (CPT^® 70553) is indicated for all members

† Postoperative MRI Brain without and with contrast (CPT^® 70553) is required (preferably within 48 hours of surgery) to quantify residual tumor volume

† MRI spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) is required for all members either preoperatively or within 28 days postoperatively

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

Members generally proceed to chemoradiotherapy within 31 days of surgical resection. All members receive adjuvant chemotherapy lasting 6 to 12 months that begins ~6 weeks after completion of chemoradiotherapy.

† MRI Brain without and with contrast (CPT^® 70553) and MRI spine without and with contrast (Cervical-CPT^® 72156, Thoracic CPT^® 72157, Lumbar-CPT^® 72158) is appropriate at the start of adjuvant chemotherapy and every 2 cycles until therapy is completed

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain
® Children age < 3 years are often treated with multiple cycles of high dose chemotherapy with autologous stem cell rescue in lieu of radiotherapy, and disease evaluations may occur prior to each cycle (every 4 to 6 weeks) if needed for response determination.

Medulloblastoma, sPNET, Pineoblastoma Surveillance:

† MRI Brain without and with contrast (CPT^® 70553) can be approved after completion of therapy every 3 months for 2 years, then every 6 months for 3 years, then annually for 10 years

† MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved after completion of therapy every 3 months for 2 years, then every 6 months for 3 years, then annually for 10 years

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

PEDONC-4.5: Atypical Teratoid/Rhabdoid Tumors (ATRT)

For this condition imaging is medically necessary based on the following criteria:

Highly aggressive tumor occurring primarily in very young children that has a clinical presentation very similar to medulloblastoma with a much higher rate of leptomeningeal spread. Metastases can occur outside the CNS, and associated tumors can also arise in the kidneys (Malignant Rhabdoid Tumor of the Kidney, MRT). Rhabdoid malignancies occurring outside the CNS should be imaged according to PEDONC-7.6: Malignant Rhabdoid Tumor of the Kidney (MRT) and Other Extracranial Sites.

Overall prognosis is poor, with < 20% of members surviving beyond 2 years from diagnosis.

Atypical Teratoid/Rhabdoid Tumor Initial Staging:

† Preoperative MRI Brain without and with contrast (CPT^® 70553) is indicated for all members

† Postoperative MRI Brain without and with contrast (CPT^® 70553) is required (preferably within 48 hours of surgery) to quantify residual tumor volume

† MRI Spine without and with contrast (Cervical-CPT^®72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) is required for all members either preoperatively or within 28 days postoperatively

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

® CT Abdomen/Pelvis with contrast (CPT^® 74177) or MRI Abdomen and Pelvis without and with contrast (CPT^® 74183 and CPT^® 72197) can be approved if a renal lesion is detected on US.
® If a renal lesion is also present, imaging guidelines for MRT should be followed
(See: PEDONC-7.6: Malignant Rhabdoid Tumor of the Kidney (MRT) and Other Extracranial Sites)

† MR Spectroscopy (CPT^® 76390) can be approved in the following circumstances:

® To evaluate a brain lesion of indeterminate nature when the MRS findings will be used to determine whether biopsy/resection can be safely postponed

Members generally proceed to induction chemotherapy shortly following surgical resection or biopsy.

† MRI Brain without and with contrast (CPT^® 70553) and MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) is appropriate after every 2 cycles of induction chemotherapy

® MRI spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain
® Children with ATRT are often treated using consolidation chemotherapy with 2 to 4 cycles of high dose chemotherapy with autologous stem cell rescue. Disease evaluation is indicated following the end of the planned stem cell rescues but may occur prior to each cycle (every 4 to 6 weeks) if needed for response determination.

† MRI Brain without and with contrast (CPT^® 70553) and MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) is appropriate at the end of all planned therapy

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain.

† MRI Brain without and with contrast (CPT^® 70553) can be approved after completion of therapy every 3 months for 2 years, then every 6 months for 3 years, then annually for 10 years

† MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved after completion of therapy every 3 months for 2 years, then every 6 months for 3 years, then annually for 10 years

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain.

PEDONC-4.6: Pineocytomas

For this condition imaging is medically necessary based on the following criteria:

Low grade malignancy that is similar in presentation to low grade glioma (LGG).

PET Brain Metabolic imaging and MR Spectroscopy do not have a defined role in the evaluation of pineocytoma.

Pineocytomas Initial Staging:

† MRI Brain without and with contrast (CPT^® 70553) is indicated for all members

† MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved for members with:

® Multicentric tumors
® Atypical histology including pineoblastoma-like elements
® Clinical signs or symptoms suggesting spinal cord involvement
® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

† Surgical resection is curative for most members. Members who have resection of the tumor can have a single MRI Brain without and with contrast (CPT^® 70553) approved following resection to establish baseline imaging and those with a complete resection should then be imaged according to surveillance guidelines

† Members with incompletely resected tumors may receive adjuvant radiation therapy and can have a single MRI Brain without and with contrast (CPT^® 70553) approved at completion of radiotherapy and should then be imaged according to surveillance guidelines

® Spinal imaging is not indicated for members without evidence of spinal cord involvement at initial diagnosis
® Spinal imaging is appropriate at completion of radiotherapy for members with measurable spinal cord disease on MRI

† MRI Brain without and with contrast (CPT^® 70553) can be approved after completion of therapy every 3 months for 1 year, then every 4 months for 1 year, then every 6 months for 1 year, then annually thereafter

† MRI Spine is not indicated during surveillance in members without prior history of spinal involvement except to evaluate symptoms suspicious for spinal cord recurrence

† For members with cord involvement at diagnosis, MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved after completion of therapy every 3 months for 1 year, then every 4 months for 1 year, then every 6 months for 1 year, then annually thereafter

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

For this condition imaging is medically necessary based on the following criteria:

More common in older school age children and younger adolescents, but can occur throughout the pediatric age range. Although leptomeningeal spread is common, prognosis is excellent due to high sensitivity to chemotherapy and radiotherapy.

Includes the following tumors:

† CNS Germinoma † Non-Germinomatous Germ Cell Tumors (NGGCT) ® Embryonal carcinoma ® Yolk sac tumor ® Choriocarcinoma ® Teratoma ® Mixed germ cell tumor

† PET Metabolic Brain imaging does not have a defined role in the evaluation of CNS GCT.

† MR Spectroscopy (CPT^® 76390) can be approved in the following circumstances:

® To evaluate a brain lesion of indeterminate nature when the MRS findings will be used to determine whether biopsy/resection can be safely postponed

† MRI Brain without and with contrast (CPT^® 70553) is indicated for all members

† MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) is indicated for all members

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

Members generally proceed to chemotherapy shortly following surgical resection or biopsy and will usually receive 2 to 4 cycles.

† MRI Brain without and with contrast (CPT^® 70553) is appropriate after every 2 cycles of induction chemotherapy

† MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) is appropriate at the end of induction chemotherapy for members with localized intracranial tumors

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain
® Spinal imaging is appropriate every 2 cycles during induction chemotherapy for members with measurable spinal cord disease on MRI

® MRI of all known sites of measurable disease can be performed prior to surgery and prior to radiotherapy, if necessary

CNS Germinoma & NGGCT Surveillance:

† MRI Brain without and with contrast (CPT^® 70553) can be approved every 3 months for 1 year, then every 4 months for 1 year, then every 6 months for 1 year, then annually until 5 years after completion of therapy

® For additional imaging guidelines for members in long term follow up after CNS tumor treatment that included radiation therapy, See PEDONC-19.3: Second Malignant Neoplasms (SMN)

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

® MRI Brain without and with contrast (CPT^® 70553)
® MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158)
¡ MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

For this condition imaging is medically necessary based on the following criteria:

Occur primarily intracranially, roughly 2/3 in the posterior fossa. Overall prognosis is very good, with supratentorial tumors faring better. Primary spinal tumors can also occur, and are more common in adult members than pediatric members.

† Surgery is the primary treatment modality. Radiotherapy +/- chemotherapy is used for:

® Incompletely resected tumors
® Anaplastic histology
® Infratentorial location

† MR Spectroscopy (CPT^® 76390) can be approved in the following circumstances:

® To evaluate a brain lesion of indeterminate nature when the MRS findings will be used to determine whether biopsy/resection can be safely postponed

† MRI Brain without and with contrast (CPT^® 70553) is indicated for all members

† MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) is indicated for all members

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

† Members who have resection of the tumor can have a single MRI Brain without and with contrast (CPT^® 70553) or MRI without and with contrast of involved spinal level(s) approved following resection to establish baseline imaging and those with a complete resection should then be imaged according to surveillance guidelines

† Members with incomplete resection or high risk histology receiving adjuvant radiation therapy can have a single MRI Brain without and with contrast (CPT^® 70553) or involved spinal level(s) approved at completion of radiotherapy and should then be imaged according to surveillance guidelines

† Members treated with chemotherapy can have MRI Brain without and with contrast (CPT^® 70553) or involved spinal level(s) approved every 2 cycles during active treatment and at the end of planned chemotherapy

† MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) is appropriate at the end of induction chemotherapy for members with localized intracranial tumors

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

† Following completion of chemotherapy some members will proceed to second-look surgery and/or radiotherapy

® MRI of all known sites of measurable disease can be performed prior to surgery and prior to radiotherapy, if necessary

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

† For members with primary intracranial ependymoma:

® MRI Brain without and with contrast (CPT^® 70553) can be approved after completion of therapy every 3 months for1 year, then every 6 months for 1 year, then annually for 10 years
® MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved annually for 2 years after completion of therapy for members with no history of spinal cord involvement
® For members with metastatic cord involvement at diagnosis, MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved after completion of therapy every 3 months for 1 year, then every 6 months for 1 year, then annually for 10 years
¡ MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

® MRI without and with contrast of the involved spinal level(s) can be approved after completion of therapy every 3 months for1 year, then every 6 months for 1 year, then annually for 10 years
® MRI Brain without and with contrast (CPT^® 70553) can be approved annually for 2 years after completion of therapy for members with no history of intracranial involvement
® For members with metastatic intracranial involvement at diagnosis, MRI Brain without and with contrast (CPT^® 70553) can be approved after completion of therapy every 3 months for 1 year, then every 6 months for 1 year, then annually for 10 years

For this condition imaging is medically necessary based on the following criteria:

Treatment principles are the same as tumors of the brain, and should follow imaging guidelines according to the specific histologic type.

Multiple spinal cord tumors should raise suspicion for neurofibromatosis.

Common histologies of primary spinal cord tumor in children include:

® Low Grade Glioma, See PEDONC-4.2: Intracranial Low Grade Glioma (LGG) for guidelines
® Ependymoma, See PEDONC-4.8: Ependymoma for guidelines
® Any type can occur, but other histologies are rare

® Entire spine imaging may be indicated based on the histologic type

® Given the rarity of primary spinal cord tumors in children, MRI Brain requests should, in general, be approved for surveillance after recent evaluation by a physician with significant training and/or experience in pediatric spinal cord tumors (most commonly a pediatric neurosurgeon or pediatric oncologist) as the need for intracranial surveillance is highly individualized

For this condition imaging is medically necessary based on the following criteria:

Imaging guidelines and treatment approaches for pediatric pituitary tumors other than craniopharyngioma are consistent with those used for adults with pituitary tumors. For these tumors follow guidelines in Adult Head Imaging Policy (Policy #151 in the Radiology Section); HD-19: Pituitary

Craniopharyngiomas are less common, accounting for 6 to 8% of pediatric CNS tumors. Most commonly affects children in the preadolescent ages. Several key imaging findings can be used to differentiate the tumors in this region including the presence of calcifications, cysts, and T1/T2 enhancement patterns in craniopharyngiomas. These are best evaluated using a COMBINATION of both MRI and CT modalities. Preoperative prediction is much more successful when BOTH modalities are obtained prior to biopsy.

Other less common tumors in the optic chiasm, sella, and suprasella region may include Germ Cell Tumors (GCT, see PEDONC-4.7) and Langerhans Cell Histiocytosis (LCH, see PEDONC-18).

† PET Brain Metabolic Imaging and MR Spectroscopy do not have a defined role in the evaluation of craniopharyngioma.

Craniopharyngioma Initial Staging:

† MRI Brain without and with contrast (CPT^® 70553) is indicated for all members

† Concurrent CT Head without contrast (CPT^® 70450) can be approved in addition to MRI if cranipharyngioma is suspected

† MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved for members with:

® Multicentric tumors
® Clinical signs or symptoms suggesting spinal cord involvement
® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

† Surgical resection is curative for many members. Members who have resection of the tumor can have a single MRI Brain without and with contrast (CPT^® 70553) approved following resection to establish baseline imaging and those with a complete resection should then be imaged according to surveillance guidelines.

† Members with incomplete resection and receiving adjuvant radiation therapy can have a single MRI Brain (CPT^® 70553) approved at completion of radiotherapy and should then be imaged according to surveillance guidelines

† Those rare members who are treated with chemotherapy can have MRI Brain without and with contrast (CPT^® 70553) approved every 2 cycles during active treatment and at the end of planned chemotherapy

® Spinal imaging is appropriate every 2 cycles during induction chemotherapy for members with measurable spinal cord disease on MRI

† MRI Brain without and with contrast (CPT^® 70553) can be approved every 3 months for 1 year, then every 4 months for 1 year, then every 6 months for 1 year, then annually until 10 years after completion of therapy as late progressions can occur

® For additional imaging guidelines for members in long term follow up after CNS tumor treatment that included radiation therapy, See PEDONC-19.3: Second Malignant Neoplasms (SMN)

PEDONC-4.11: Primary CNS Lymphoma

For this condition imaging is medically necessary based on the following criteria:

Primary CNS lymphoma is a solitary or multifocal mass occurring in the brain without evidence of systemic (bone marrow or lymph node) involvement. Usually associated with immunodeficiency, this is a very rare entity in pediatrics accounting for < 0.1% of pediatric malignancies, so age-specific guidelines have not been established.

Primary CNS lymphoma imaging indications in pediatric members are identical to those for adult members. See Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-2.7: CNS Lymphoma for imaging guidelines.

CNS lymphomas also involving bone marrow and/or lymph nodes should be imaged according to: PEDONC-5.3: Pediatric Aggressive Mature B-Cell Non-Hodgkin Lymphomas (NHL).

PEDONC-4.12: Meningiomas

For this condition imaging is medically necessary based on the following criteria:

Account for 1 to 3% of pediatric CNS tumors. Usually associated with neurofibromatosis type 2 (NF-2) or prior therapeutic radiation exposure to the brain. Lifetime risk may be as high as 20% for young children receiving whole brain radiotherapy, most commonly occurring 15 to 20 years after radiation exposure.

Meningioma imaging indications in pediatric members are identical to those for adult members. See Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-2.8: CNS Meningioma for imaging guidelines.

For this condition imaging is medically necessary based on the following criteria:

As a group these account for 1 to 4% of pediatric CNS tumors, and 70% of choroid plexus tumors present within the first 2 years of life.

† Includes the following tumors:

® Choroid plexus papilloma
® Choroid plexus adenoma, or atypical choroid plexus papilloma
® Choroid plexus carcinoma

† MR Spectroscopy (CPT^® 76390) can be approved in the following circumstances:

® To evaluate a brain lesion of indeterminate nature when the MRS findings will be used to determine whether biopsy/resection can be safely postponed

Choroid plexus papillomas outnumber other choroid plexus tumors by 4 to 5 times. These ventricular tumors commonly present with hydrocephalus caused by increased CSF production, resulting in signs of increased intracranial pressure. Appearance on MRI Brain without and with contrast (CPT^® 70553) is typical, and they are usually treated by excision.

† Regrowth is rare, but repeat MRI Brain without and with contrast (CPT^® 70553) is indicated if return of hydrocephalus is suspected or seen on CT imaging

Choroid Plexus Adenoma or Atypical Choroid Plexus Papilloma

These are extremely rare tumors with features midway in the malignant spectrum between papillomas and carcinomas. They are more prone to local invasion, but rarely to metastasis. Presenting symptoms are similar to papillomas. Appearance on MRI Brain with and without contrast (CPT^® 70553) is typical, and they are usually treated by excision.

† Spinal imaging may be approved if requested at initial diagnosis

† Regrowth is rare, but repeat MRI Brain without and with contrast is indicated if return of hydrocephalus is suspected or Seen on CT imaging

Choroid Plexus Carcinoma

This is a very aggressive malignancy, with high rates of metastasis to other parts of the CNS. Prognosis is significantly less favorable than for papillomas with overall survival rates of 35 to 40%. Overall incidence of metastases in choroid plexus carcinoma is 12–50%, which is associated with a worse outcome. TP53 mutations and alternative lengthening telomeres (ALT) are common in members with choroid plexus carcinoma.

Choroid Plexus Carcinoma Initial Staging:

† MRI Brain without and with contrast (CPT^® 70553) is indicated for all members

† MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) is indicated for all members

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

† Surgical gross total resection is curative for many members. Members who have gross or subtotal resection of the tumor can have a single MRI Brain without and with contrast (CPT^® 70553) approved following resection to establish baseline imaging.

® Members with confirmed gross total resection should then be imaged according to surveillance guidelines.

† Members treated with chemotherapy can have MRI Brain without and with contrast (CPT^® 70553) approved every 2 cycles during active treatment and at the end of planned chemotherapy

† MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) is appropriate at the end of chemotherapy for members with localized intracranial tumors

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain
® Spinal imaging is appropriate every 2 cycles during chemotherapy for members with measurable spinal cord disease on MRI

® MRI of all known sites of measurable disease can be performed prior to surgery and prior to radiotherapy, if necessary

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

† MRI Brain without and with contrast (CPT^® 70553) can be approved every 4 months for 3 years, then every 6 months for 2 years after completion of therapy

® For additional imaging guidelines for members in long term follow up after CNS tumor treatment that included radiation therapy, See PEDONC-19.3: Second Malignant Neoplasms (SMN)

† For members with cord involvement at diagnosis, MRI Spine without and with contrast (Cervical-CPT^® 72156, Thoracic-CPT^® 72157, Lumbar-CPT^® 72158) can be approved every 4 months for 3 years, then every 6 months for 2 years after completion of therapy

® MRI Spine with contrast only (Cervical-CPT^® 72142, Thoracic-CPT^® 72147, Lumbar-CPT^® 72149) can be approved if being performed immediately following a contrast-enhanced MRI Brain

References

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2. Chintagumpala MM, Paulino A, Panigraphy A, et al. Embryonal and pineal region tumors. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:671-699.
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15. Kruer MC, Kaplan AM, Etzl MM Jr, et al. The value of positron emission tomography and proliferation index in predicting progression in low-grade astrocytomas of childhood. J Neurooncol. 2009;95(2):239-245. doi:10.1007/s11060-009-9922-4.
16. Chamdine O, Broniscer A, Wu S, Gajjar A, Qaddoumi I. Metastatic low-grade gliomas in children: 20 years’ experience at St. Jude Children’s Research Hospital. Pediatr Blood Cancer. 2016;63(1):62-70. doi:10.1002/pbc.25731.
17. Chalil A, Ramaswamy V. Low grade gliomas in children. J Child Neurol. 2016;31(4):517-522. doi:10.1177/0883073815599259.
18. Krishnatry R, Zhukova N, Stucklin ASG, et al. Clinical and treatment factors determining long-term outcomes for adult survivors of childhood low-grade glioma: a population-based study. Cancer. 2016;122(8):1261-1269. doi:10.1002/cncr.29907.
19. Ullrich NJ. Neurocutaneous Syndromes and brain tumors. J Child Neurol. 2016;31(12):1399-1411. doi:10.1177/0883073815604220.
20. Campian J, Gutmann DH. CNS tumors in neurofibromatosis. J Clin Oncol. 2017; 35(21):2378-2385. doi:10.1200/JCO.2016.71.7199.
21. Morris EB, Gajjar A, Okuma JO, et al. Survival and late mortality in long-term survivors of pediatric CNS tumors. J Clin Oncol. 2007;25(12):1532-1538. doi:10.1200/JCO.2006.09.8194.
22. Karthigeyan M, Gupta K, Salunke P. Pediatric central neurocytoma: a short series with literature review. J Child Neurol. 2016;32(1):53-59. Accessed January 3, 2018 doi:10.1177/0883073816668994.
23. Tisnado J, Young R, Peck KK, et al. Conventional and advanced imaging of diffuse intrinsic pontine glioma. J Child Neurol. 2016;31(12):1386-1393. doi:10.1177/0883073816634855.
24. Greenfield JP, Heredia AC, George E, Keiran MW, Morales La Madrid A. Gliomatosis cerebri: a consensus summary report from the First International Gliomatosis cerebri Group Meeting, March 26-27, 2015, Paris, France. Pediatr Blood Cancer. 2016;63(12):2072-2077. doi:10.1002/pbc.26169.
25. Zaky W, Dhall G, Ji L, et al. Intensive induction chemotherapy followed by myeloablative chemotherapy with autologous hematopoietic progenitor cell rescue for young children newly-diagnosed with central nervous system atypical teratoid/rhabdoid tumors: the head start III experience. Pediatr Blood Cancer. 2014;61(1):95-101. doi:10.1002/pbc.24648.
26. Gururangan S, Hwang E, Herndon JE II, et al. [18F]Fluorodeoxyglucose-Positron Emission Tomography in patients with medulloblastoma. Neurosurgery. 2004;55(6):1280-1289. doi:10.1227/01.NEU.0000143027.41632.2B.
27. Millard NE and DeBraganca KC. Medulloblastoma. J Child Neurol. 2016;31(12):1341-1353. doi:10.1177/0883073815600866.
28. Koschmann C, Bloom K, Upadhyaya S, Geyer JR. Survival after relapse of medulloblastoma. J Pediatr Hematol. Oncol. 2016;38(4):269-273. doi:10.1097/MPH.0000000000000547.
29. Martinex S, Khakoo Y, Giheeney S, et al. Marker (+) CNS germ cell tumors in remission: are surveillance MRI scans necessary? Pediatr Blood Cancer. 2014;61(5):853-854. Accessed January 3, 2018. doi:10.1002/pbc.24888.
30. Goldman S, Bouffet E, Fisher PG, et al. Phase II trial assessing the ability of neoadjuvant chemotherapy with or without second-look surgery to eliminate measurable disease for nongerminomatous germ cell tumors: a children’s oncology group study. J Clin Oncol. 2015;33(22):2464-2471. doi:10.1200/JCO.2014.59.5132.
31. Abu Arja MH, Bouffet E, Finlay JL, AbdelBaki MS. Critical review of the management of primary central nervous system germ cell tumors. Pediatr Blood Cancer. 2019;66:e27658. doi:10.1002/pbc.27658.
32. Vitanza NA, Partap S. Pediatric Ependymoma. J Child Neurol. 2016;31(12):1354-1366. doi:10.1177/0883073815610428
33. Norris GA, Garcia J, Hankinson TC, et al. Diagnostic accuracy of neuroimaging in pediatric optic chiasm/suprasellar tumors. Pediatr Blood Cancer. 2019;66:e27860. doi:10.1002/pbc.27680.
34. McCrea HJ, George E, Settler A, et al. Pediatric suprasellar tumors. J Child Neurol. 2016;31(12):1367-1376. doi:10.1177/0883073815620671.
35. Grommes C, DeAngelis LM. Primary CNS lymphoma. J Clin Oncol. 2017;35(21):2410-2418. doi:10.1200/JCO.2017.72.7602.
36. Larrew T, Eskandari R. Pediatric intraparenchymal meningioma, case report and comparative review. Pediatr Neurosurg. 2016;51(2):83-86. doi:10.1159/000441008.
37. Li Z, Li H, Wang S, et al. Pediatric skull base meningiomas: clinical features and surgical outcomes. J Child Neurol. 2016;31(14):1523-1527. doi:10.1177/0883073816664669.
38. Horská A, Barker PB. Imaging of brain tumors: MR Spectroscopy and metabolic imaging. Neuroimaging Clin N Am. 2010;20(3):293-310. doi:10.1016/j.nic.2010.04.003.
39. Sundgren PC. MR Spectroscopy in radiation injury. AJNR Am J Neuroradiol. 2009;30(8):1469-1476. doi:10.3174/ajnr.A1580.
40. Zaky W, Dhall G, Khatua S, et al. Choroid plexus carcinoma in children: the head start experience. Pediatr Blood Cancer. 2015;62(5):784-789. doi:10.1002/pbc.25436.
41. Zaky W, Finlay JL, Pediatric choroid plexus carcinoma: Biologically and clinically in need of new perspectives, Pediatr Blood Cancer 2018;65:e27031. doi:10.1002/pbc.27031.
42. Kramer K. Rare primary central nervous system tumors encountered in pediatrics. J Child Neurol. 2016;31(12):1394-1398. doi:10.1177/0883073815627878.

For this condition imaging is medically necessary based on the following criteria:

† Lymphoma mostly commonly involves the lymph nodes (LNs). However, lymphoma can also arise from primary lymphoid tissues (bone marrow or thymus) or various secondary lymphoid tissues (spleen, mucosa-associated lymphoid tissue) or non-lymphoid organs (skin, bone, brain, lungs, liver, salivary glands, etc).

† Pediatric lymphomas are generally Hodgkin Lymphomas, Aggressive B-Cell Non-Hodgkin Lymphomas, Lymphoblastic Lymphomas, or Anaplastic Large Cell Lymphomas

† Members with Lymphoblastic Lymphoma (even those with bulky nodal disease) are treated using the leukemia treatment plan appropriate to the cell type (B or T cell). These members should be imaged using guidelines in PEDONC-3.2: Acute Lymphoblastic Leukemia.

† Other histologies are rare in pediatric members, and should be imaged according to the following guidelines:

® Follicular lymphoma: Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-27.3: Follicular Lymphoma
® Marginal zone or MALT lymphomas: Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-27.4: Marginal Zone Lymphomas
® Mantle cell lymphomas: Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-27.5: Mantle Cell Lymphoma
® Cutaneous lymphomas: Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-27.8: Cutaneous Lymphomas and T Cell Lymphomas
¡ Exception: Cutaneous B-Lymphoblastic Lymphoma should be imaged using guidelines in PEDONC-3.2: Acute Lymphoblastic Leukemia
® Castleman’s Disease: Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-31.11: Castleman’s Disease (Unicentric and Multicentric)

® Noncontrast CT imaging has not been shown to be beneficial in the management of pediatric lymphomas
® Given the limited utility of noncontrast CT imaging in pediatric lymphomas, MRI without or without and with contrast is recommended in place of CT for members who cannot tolerate CT contrast due to allergy or impaired renal function

® Bone scan is inferior to MRI for evaluation of known or suspected bone metastases in lymphoma

® CT Head with (CPT^® 70460) or without and with contrast (CPT^® 70470) can be approved when MRI is contraindicated

For this condition imaging is medically necessary based on the following criteria:

Pediatric Hodgkin Lymphoma Initial Staging:

† All members should undergo CT Neck (CPT^® 70491), Chest (CPT^® 71260), Abdomen/Pelvis (CPT^® 74177), and CT with contrast or MRI without and with contrast of any other symptomatic body area is indicated for all members (See PEDONC-5.1: Pediatric Lymphoma – General Considerations) as pediatric members have a high rate of neck and Waldeyer’s ring involvement with Hodgkin Lymphoma

† PET/CT (CPT^® 78815) is indicated for initial staging of all members, and can be performed prior to biopsy if necessary for member scheduling

® Whole body PET/CT (CPT^® 78816) may be approved if there is clinical suspicion of skull or distal lower extremity involvement

Pediatric Hodgkin Lymphoma Treatment Response:

† Restaging for treatment response can be performed as often as every 2 cycles of chemotherapy

† Both CT of Neck (CPT^® 70491), Chest (CPT^® 71260), and Abdomen/Pelvis (CPT^® 74177) and other previously involved areas and PET/CT (CPT^® 78815) can be approved during early treatment response evaluations as decisions about chemotherapy drug selection and radiation treatment are frequently made based on both anatomic (CT-based) and metabolic (PET/CT-based) responses.

® For members with low risk (stage IA or IIA) mixed cellularity Hodgkin lymphoma, PET/CT can be performed for treatment response after cycles 1 and 3 instead of cycles 2 and 4

Pediatric Hodgkin Lymphoma Surveillance:

Most members experiencing recurrence are detected based on physical findings, and frequent CT surveillance imaging of Hodgkin Lymphoma after completion of therapy does not improve post-recurrence overall survival.

† CT of the Neck (CPT^® 70491), Chest (CPT^® 71260), Abdomen/Pelvis (CPT^® 74177) and other previously involved or currently symptomatic areas OR PET/CT (CPT^® 78815 or 78816) should be approved for any member with clinical symptoms suggesting recurrence

† Members with stage I or II HL:

® CT of the Neck/Chest (CPT^® 70491 and CPT^® 71260) and other previously involved areas at 6 months and 12 months after completing therapy
® Surveillance at other time points from the end of therapy should use physical exam and CXR only

® CT of the Neck (CPT^® 70491), Chest (CPT^® 71260), and Abdomen/Pelvis (CPT^® 74177) and other previously involved areas at 6 months and 12 months after completing therapy
® Surveillance at other time points from the end of therapy should use physical exam and CXR only

® CT of the Neck/Chest/Abdomen/Pelvis every 3 months for 1 year after completing therapy for recurrence

For this condition imaging is medically necessary based on the following criteria:

† Aggressive mature B-Cell NHL includes all of the following diagnoses, all of which should be imaged according to this section:

® Burkitt’s lymphoma/leukemia (BL)
® Diffuse Large B-Cell Lymphoma (DLBCL)
® Primary Mediastinal Large B-Cell Lymphoma (PMBCL)
® Post-transplant Lymphoproliferative Disorder (PTLD)
¡ Most commonly occurs following solid organ or stem cell transplantation
® Viral-associated lymphoproliferative disorders
¡ Most commonly occurs following hematopoietic stem cell transplantation or in members with primary immunodeficiency

† CT of the Neck (CPT^® 70491), Chest (CPT^® 71260), and Abdomen/Pelvis (CPT^® 74177) and CT with contrast or MRI without and with contrast any other symptomatic body area is indicated for all members (See PEDONC-5.1: Pediatric Lymphoma –General Considerations)

† MRI Brain without and with contrast (CPT^® 70553) is indicated if symptoms or extent of disease suggest intracranial extension (skull base involvement, for example) or metastasis

† PET/CT (CPT^® 78815) is indicated for initial staging for all members

® Whole body PET/CT (CPT^® 78816) may be approved if there is clinical suspicion of skull or distal lower extremity involvement.
® Due to the extremely aggressive nature of this group of tumors (the doubling time can be as short as 8 hours) it may not be possible to obtain PET/CT prior to therapy initiation. PET/CT should be approved for treatment response in these cases as these lymphomas are nearly universally FDG-avid.

† Initial treatment is usually 7 days of low intensity therapy, with early response evaluation determining next steps in therapy using CT with contrast or MRI without and with contrast of previously involved areas performed around day 6

® Members are customarily still inpatient for this evaluation so outpatient requests should be rare for this time point

† Once a particular member has a negative PET/CT (either Deauville or Lugano 1, 2 or 3 as reported in formal radiology interpretation), all subsequent treatment response evaluations should use CT imaging only, including end of therapy evaluation

® PET/CT may be indicated to assess disease activity in inconclusive residual masses seen on conventional imaging

Routine asymptomatic surveillance with advanced imaging has not been found to impact member outcomes as the majority of these members present clinically at relapse due to the highly aggressive nature of these lymphomas.

† CXR and Abdominal (CPT^® 76700) and Pelvic (CPT^® 76856) ultrasound are sufficient to follow asymptomatic members with residual masses in the chest or abdomen/pelvis. Surveillance imaging with CT or MRI has not been shown to improve member outcomes following recurrence and is not the standard of care.

† CT of the Neck (CPT^® 70491), Chest (CPT^® 71260), Abdomen/Pelvis (CPT^® 74177) and other previously involved or currently symptomatic areas OR PET/CT (CPT^® 78815 or 78816) should be approved for any member with clinical symptoms or laboratory findings suggesting recurrence.

® PET/CT (CPT^® 78815) can be approved for suspected PTLD recurrence with documentation of new palpable nodes, rising LDH, or rising quantitative EBV PCR

For this condition imaging is medically necessary based on the following criteria:

Similar in presentation to Hodgkin Lymphoma, and may be indistinguishable until immunocytology and molecular studies are complete.

Anaplastic Large Cell Lymphoma Initial Staging:

† All members should undergo CT of the Neck/Chest/Abdomen/Pelvis (CPT^® 70491, CPT^® 71260, and CPT^® 74177) and CT with contrast or MRI without and with contrast any other symptomatic body area is indicated for all members (See PEDONC-5.1: Pediatric Lymphoma – General Considerations)

† PET/CT (CPT^® 78815) is indicated for initial staging of all members and can be performed prior to biopsy if necessary for member scheduling.

® Whole body PET/CT (CPT^® 78816) may be approved if there is clinical suspicion of skull or distal lower extremity involvement.

† Bone scan (See PEDONC-1.3: Modality General Considerations) is indicated for members with bony primary tumors or metastatic disease

Anaplastic Large Cell Lymphoma Treatment Response:

† Restaging for treatment response using CT with contrast or MRI without and with contrast of previously involved areas (should be same modality as initial diagnosis if possible) should be performed at the end of induction chemotherapy (commonly 4 to 6 weeks)

† For members treated with cytotoxic chemotherapy, either CT of previously involved areas or PET/CT may be approved for treatment response as often as every 2 cycles of chemotherapy as decisions about chemotherapy drug selection and radiation treatment can be made based on either anatomic or metabolic responses.

® If CT is performed for primary treatment response, PET/CT can be approved to clarify inconclusive findings detected on conventional imaging
® If PET/CT is performed for primary treatment response, CT or MRI can be approved to clarify inconclusive findings detected on PET imaging

Anaplastic Large Cell Lymphoma Surveillance:

† CT of the Neck (CPT^® 70491), Chest (CPT^® 71260), Abdomen/Pelvis (CPT^® 74177) and other previously involved or currently symptomatic areas should be approved for any member with clinical symptoms suggesting recurrence.

† CT with contrast or MRI without and with contrast of all previously involved areas is indicated at 3, 6, 12, and 18 months after therapy is completed.

† Bone scan (See PEDONC-1.3: Modality General Considerations) is indicated at 3, 6, 12, and 18 months after therapy is completed for members with bony primary tumors or metastatic disease

† PET/CT is not indicated for surveillance, but can be approved to clarify inconclusive findings on conventional imaging to evaluate the need for biopsy to establish recurrence. These requests should be forwarded for Medical Director review.

References

1. Metzger ML, Krasin MJ, Choi JK, et al. Hodgkin lymphoma. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:568-586.
2. Allen CE, Kamdar KY, Bollard CM, et al. Malignant Non-Hodgkin lymphomas in Children. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:587-603.
3. Hayashi RJ, Wistinghausen B, Shiramizu B. Lymphoproliferative Disorders and Malignancies Related To Immunodeficiencies. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7^th ed. Philadelphia, PA: Wolters Kluwer; 2016:604-616.
4. Alexander S, Ferrando AA. Pediatric lymphoma. In: Orkin SH, Fisher DE, Ginsburg D, Look AT, Lux SE, Nathan DG, eds. Nathan and Oski’s Hematology and Oncology of Infancy and Childhood. 8^th ed. Philadelphia, PA: Elsevier Saunders; 2015:1626-1671.
5. Lee Chong A, Grant RM, Ahmed BA, Thomas KE, Connolly BL, Greenberg M. Imaging in pediatric patients: time to think again about surveillance. Pediatr Blood Cancer. 2010;55(3):407-413. doi:10.1002/pbc.22575.
6. Uslu L, Doing J, Link M, Rosenberg J, Quon A, Daldrup-Link H. Value of 18F-FDG-PET and PET/CT for evaluation of pediatric malignancies. J Nucl Med. 2015;56(2):274-286. doi:10.2967/jnumed.114.146290.
7. Ceppi F, Pope E, Ngan B, Abla O. Primary cutaneous lymphomas in children and adolescents. Pediatr Blood Cancer. 2016;63(11):1886-1894. doi:10.1002/pbc.26076.
8. Flerlage JE, Kelly KM, Beishuizen A, et al. Staging evaluation and response criteria harmonization (SEARCH) for childhood, adolescent, and young adult Hodgkin Lymphoma (CAYAHL): Methodology statement. Pediatr Blood Cancer. 2017;64(7):e26421. doi:10.1002/pbc.26421.
9. Friedman DL, Chen L, Wolden S, et al. Dose-intensive response-based chemotherapy and radiation therapy for children and adolescents with newly diagnosed intermediate-risk Hodgkin lymphoma: a report from the Children’s Oncology Group Study AHOD0031. J Clin Oncol. 2014;32(32):3651-3658. doi:10.1200/JCO.2013.52.5410.
10. Schwartz CL, Chen L, McCarten K, et al. Childhood Hodgkin International Prognostic Score (CHIPS) predicts event-free survival in Hodgkin lymphoma: a report from the Children’s Oncology Group. Pediatr Blood Cancer. 2017;64(4):e26278. doi:10.1002/pbc.26278.
11. Friedmann AM, Wolfson JA, Hudson MM, et al. Relapse after treatment of Hodgkin lymphoma: outcome and role of surveillance after therapy. Pediatr Blood Cancer. 2013;60(9):1458-1463. doi:10.1002/pbc.24568.
12. Mauz-Körholz C, Metzger ML, Kelly KM, et al. Pediatric Hodgkin lymphoma. J Clin Oncol. 2015;33(27):2975-2985. doi:10.1200/JCO.2014.59.4853.
13. Voss SD, Chen L, Constine LS, et al. Surveillance computed tomography imaging and detection of relapse in intermediate- and advanced-stage pediatric Hodgkin’s lymphoma: A report from the Children’s Oncology Group. J Clin Oncol. 2012;30(21):2635-2640. doi:10.1200/JCO.2011.40.7841.
14. Voss SD. Surveillance Imaging in Pediatric Hodgkin Lymphoma. Curr Hematol Malig Rep. 2013;8(3):218-225. doi:10.1007/s11899-013-0168-z.
15. Appel BE, Chen L, Buxton AB, et al. Minimal treatment of low-risk, pediatric lymphocyte-predominant Hodgkin lymphoma: a report from the Children’s Oncology Group. J Clin Oncol. 2016;34(20):2372-2379. doi:10.1200/JCO.2015.65.3469.
16. Rosolen A, Perkins SL, Pinkerton CR, et al. Revised international pediatric non-Hodgkin lymphoma staging system. J Clin Oncol. 2015;33:2112-2118. doi:10.1200/JCO.2014.59.7203.
17. Sandlund JT, Guillerman RP, Perkins SL, et al. International pediatric Non-Hodgkin lymphoma response criteria. J Clin Oncol. 2015;33(18):2106-2111. doi:10.1200/JCO.2014.59.0745.
18. Eissa HM, Allen CE, Kamdar K, et al. Pediatric Burkitt’s lymphoma and Diffuse B-cell lymphoma: are surveillance scans required? Pediatr Hematol Oncol. 2014;31(3):253-257. doi:10.3109/08880018.2013.834400.
19. Minard-Colin V, Brugieres L, Reiter A, et al. Non-Hodgkin lymphoma in children and adolescents: progress through effective collaboration, current knowledge, and challenges ahead. J Clin Oncol. 2015;33(27):2963-2974. doi:10.1200/JCO.2014.59.5827.
20. Dierickx D, Tousseym T, and Gheysens O. How I treat posttransplant lymphoproliferative disorders. Blood. 2015;126(20):2274-2283. doi:10.1182/blood-2015-05-615872.
21. Vali R, Punnett A, Bajno L, Moineddin R, Shammas A. The value of ¹⁸F-FDG PET in pediatric patients with post-transplant lymphoproliferative disorder at initial diagnosis. Pediatr Transplant. 2015;19(8):932-939. doi:10.1111/petr.12611.
22. Hapgood G and Savage KJ. The biology and management of systemic anaplastic large cell lymphoma. Blood. 2015;126(1):17-25. doi:10.1182/blood-2014-10-567461.
23. Kempf W, Kazakov DV, Belousova IE, Mitteldorf C, Kerl K. Pediatric cutaneous lymphomas: a review and comparison with adult counterparts. J Eur Acad Dermatol Venereol. 2015;29(9):1696-1709. doi:10.1111/jdv.13044.
24. Hochberg J, Flower A, Brugieres L, Cairo MS. NHL in adolescents and young adults: a unique population. Pediatr Blood Cancer. 2018;65:e27073. doi.org/10.1002/pbc.27073.
25. Buhtoiarov IN. Pediatric lymphoma. Pediatr In Rev. 2017;38(9):410-423. doi:10.1542/pir.2016-0152.

PEDONC-6: Neuroblastoma

PEDONC-6.1: Neuroblastoma – General Considerations

PEDONC-6.2: Staging and Risk Grouping – Neuroblastoma

PEDONC-6.3: Neuroblastoma – Initial Staging

PEDONC-6.4: Neuroblastoma – Treatment Response Imaging (Risk Group Dependent)

PEDONC-6.5: Neuroblastoma – Surveillance Imaging (Risk Group Dependent)

PEDONC-6.1: Neuroblastoma – General Considerations

For this condition imaging is medically necessary based on the following criteria:

Note: Some payors consider PET to be investigational for the treatment of neuroblastoma, and those coverage policies may supersede the recommendations for PET in this section.

Neuroblastoma is the most common extracranial solid tumor of childhood, and generally arises from the adrenal gland or along the sympathetic chain. Neuroblastoma is divided into very low, low, intermediate, and high risk disease based on International Neuroblastoma Risk Group (INRG) Staging System (see: PEDONC-6.5). The treatment approaches for each risk group vary widely and have distinct imaging strategies.

90 to 95% of neuroblastomas secrete homovanillic acid (HVA) and vannilylmandelic acid (VMA) in the urine, and urine HVA/VMA should be performed at every disease evaluation for members with positive HVA or VMA at diagnosis.

† Esthesioneuroblastoma should be imaged according to guidelines in Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-3: Squamous Cell Carcinomas Of The Head And Neck

† PET imaging is rarely indicated in neuroblastoma, but can be approved in the following situations:

® Members with MIBG-negativity documented at initial diagnosis
¡ For these members, MIBG should not be repeated and whole body PET (CPT^® 78816) may be performed rather than MIBG for metabolic tumor assessment.
¡ Members who are MIBG positive at diagnosis and then become MIBG negative in response to treatment should continue to use MIBG (CPT^® 78800, 78801, 78802, 78803, or 78804) for metabolic imaging indications
® For all members, PET may be approved at major decision points such as hematopoietic stem cell transplantation or surgery if MIBG and CT/MRI findings are inconclusive
® Members currently receiving medications that may interfere with MIBG uptake that cannot safely be discontinued prior to imaging, including:
¡ Tricyclic antidepressants (amitriptyline, imipramine, etc.)
¡ Selective serotonin reuptake inhibitors (SSRI’s, sertraline, paroxetine, escitolapram, etc.)
¡ Neuroleptics (risperidone, haloperidol, etc.)
¡ Antihypertensive drugs (alpha or beta blockers, calcium channel blockers)
¡ Decongestants (phenylephrine, ephedrine, pseudoephedrine)
¡ Stimulants (methylphenidate, dextroamphetamine, etc.)
¡ PET should only be approved for this indication when specific documentation of the medication interaction is included with the current PET imaging request. These requests will be forwarded for Medical Director review.

For this condition imaging is medically necessary based on the following criteria:

† Most recent treatment protocols are using the recently validated International Neuroblastoma Risk Group (INRG) staging system, which is primarily defined by the complexity of local tumor extension and the presence or absence of distant metastases:

® L1: Localized tumor not involving vital structures as defined by the list of image-defined risk factors and confined to one body compartment
¡ Image-defined risk factors include a list of specific imaging findings defining members less likely to be candidates for complete surgical resection
¡ These risk factors involve the encasement of major blood vessels, airway, skull base, costovertebral junction, brachial plexus, spinal canal, or major organs or structures
® L2: Locoregional tumor with presence of one or more image-defined risk factors
® M: Distant metastatic disease (except stage MS)
® MS: Metastatic disease in children younger than 18 months with metastases confined to skin, liver, and/or bone marrow with < 10% involvement (MIBG must be negative in bone and bone marrow)

For this condition imaging is medically necessary based on the following criteria:

† One of the following imaging groups for all members:

® CT with contrast of the Neck/Chest/Abdomen/Pelvis (CPT^® 70491, 71260, and 74177) OR
® MRI without and with contrast of the Neck/Chest/Abdomen/Pelvis (CPT^® 70543, 71552, 74183, and 72197)

† Metabolic imaging in neuroblastoma:

® Adrenal nuclear imaging (CPT^® 78075) can be approved for evaluation of suspected adrenal neuroblastoma, ganglioneuroblastoma, or ganglioneuroma when CT or MRI is inconclusive
® ¹²³I-metaiodobenzylguanidine (MIBG - CPT^® 78800, 78801, 78802, 78803, or 78804) scintigraphy is the preferred metabolic imaging for neuroblastoma and is positive in 90 to 95% of neuroblastomas.
¡ MIBG provides superior sensitivity and sensitivity for detecting viable osseous disease compared with bone scintigraphy so technetium bone scan is not necessary when MIBG is utilized
® Most MIBG imaging studies are SPECT/CT studies using CT for localization only. Separate diagnostic CT codes should not be approved for this purpose.
® Occasionally MIBG cannot be performed prior to initiation of therapy. In this circumstance MIBG should be completed within 3 weeks of therapy initiation as the reduction in MIBG avidity in response to chemotherapy is not immediate. Inability to complete MIBG before starting therapy is not an indication to approve PET imaging.
® PET imaging is inferior to MIBG in neuroblastoma, and should not be used unless one of the exceptions stated in section PEDONC-6.1 is present

® MRI Brain of asymptomatic members with no history of brain metastases is not indicated for neuroblastoma.

For this condition imaging is medically necessary based on the following criteria:

Risk grouping will not be known at the time of initial staging, but is critical for all imaging decisions after initial staging is complete. The treating oncologist should always know the member’s risk grouping. It is not possible to establish the appropriate imaging plan for a neuroblastoma member without knowing his/her risk group.

All Very Low Risk and Low Risk Neuroblastoma Not Receiving Chemotherapy:

† All members can have CT with contrast or MRI without and with contrast of the primary tumor site 6 to 8 weeks after diagnosis to determine if additional treatment is necessary.

® Ultrasound may be used in place of CT or MRI to avoid radiation and/or anesthesia exposure in low risk members

All Intermediate Risk Neuroblastoma and Very Low Risk or Low Risk Neuroblastoma Receiving Chemotherapy:

Members generally receive 2 to 12 cycles of moderate-intensity chemotherapy depending on response to treatment.

Surgical resection may occur prior to or following chemotherapy depending on disease stage. Restaging prior to surgery is appropriate.

† Treatment response assessment can be approved as often as every 2 cycles of chemotherapy (~every 6 weeks and at the end of planned treatment) and includes:

® CT with contrast of the Chest/Abdomen/Pelvis (CPT^® 71260, and CPT^® 74177) or MRI without and with contrast, (CPT^® 71552, CPT^® 74183, and CPT^® 72197) and other sites with prior measurable disease
® Urine HVA/VMA (if positive at diagnosis)
® Bone marrow aspiration/biopsy if positive at diagnosis

High Risk Neuroblastoma:

This group of members receives highly aggressive therapy using sequential chemotherapy, surgery, high dose chemotherapy with stem cell rescue, radiotherapy, monoclonal antibody (mAb) immunotherapy, and biologic therapy.

† Treatment response assessment can be approved as often as every 2 cycles of chemotherapy, mAb, or biologic therapy (~every 6 weeks) and includes:

® CT with contrast, of the Chest/Abdomen/Pelvis (CPT^® 71260, and CPT^® 74177) or MRI without and with contrast, (CPT^® 71552, CPT^® 74183, and CPT^® 72197) and other sites with prior measurable disease
® Urine HVA/VMA (if positive at diagnosis)
® Bone marrow aspiration/biopsy if positive at diagnosis
® MIBG scan (CPT^® 78800, 78801, 78802, 78803, or 78804)
¡ ¹²³I-MIBG scan is also indicated following ¹³¹I-MIBG therapy, but FDG-PET cannot be used after ¹³¹I-MIBG therapy

† More frequent imaging can be approved around the time of surgery if needed for preoperative planning

PEDONC-6.5: Neuroblastoma – Surveillance Imaging (Risk Group Dependent)

For this condition imaging is medically necessary based on the following criteria:

Very Low Risk and Low Risk Neuroblastoma:

† Urine HVA/VMA (if positive at diagnosis) at 1, 2, 3, 6, 9, 12, 18, 24, 36, 48, and 60 months after surgery

† CT with contrast or MRI without and with contrast of the primary tumor site 3, 6, 9, 12, 18, 24, and 36 months after surgery. If negative at 36 months, no further advanced imaging is necessary.

® Ultrasound may be sufficient to evaluate the primary tumor site for certain members and may be approved if requested to replace CT or MRI.

† CT Chest is not indicated in asymptomatic surveillance imaging of neuroblastoma members with no prior history of thoracic disease

Intermediate Risk Neuroblastoma:

† Urine HVA/VMA (if positive at diagnosis) every month until 12 months after completion of therapy, then at 14, 16, 18, 21, 24, 30, and 36 months after completion of therapy, then annually until 10 years after completion of therapy

† CT with contrast or MRI without and with contrast of the primary tumor and known metastatic sites at 3, 6, 9, 12, 18, 24, and 36 months after completion of therapy. If negative at 36 months, no further advanced imaging is necessary.

® Ultrasound may be sufficient to evaluate the primary tumor site for certain members and may be approved if requested to replace CT or MR

® If negative at 36 months, no further MIBG imaging is necessary.
® For all other intermediate risk neuroblastoma members, MIBG (or PET, if MIBG-negative at initial diagnosis) during surveillance is not indicated.

High Risk Neuroblastoma:

† Urine HVA/VMA (if positive at diagnosis) at 3, 6, 9, 12, 18, 24, 30, 36, 42, 48, 54, and 60 months after completion of therapy, then annually until 10 years after completion of therapy.

† CT with contrast or MRI without and with contrast of the primary tumor site at 3, 6, 9, 12, 18, 24, 30, 36, 42, 48, 54, and 60 months, then annually until 10 years after completion of therapy. If negative at 10 years, no further advanced imaging is necessary.

† MIBG scan (CPT^® 78800, 78801, 78802, 78803, or 78804) at 3, 6, 9, 12, 18, 24, 30, and 36 months after completion of therapy. If negative at 36 months, no further MIBG or PET imaging is necessary.

® Early detection of recurrence with ¹²³I-MIBG has been shown to improve post-relapse outcomes in high risk neuroblastoma

† For members with suspected recurrence:

® CT with contrast, of the Chest/Abdomen/Pelvis (CPT^® 71260, and CPT^® 74177) or MRI without and with contrast, (CPT^® 71552, CPT^® 74183, and CPT^® 72197) and other sites of prior measurable disease or current symptoms
® MIBG scan (CPT^® 78800, 78801, 78802, 78803, or 78804)
® Urine HVA/VMA

For this condition imaging is medically necessary based on the following criteria:

Note: Some payors consider PET imaging to be investigational for the treatment of Wilms tumor and other kidney tumors, and those coverage policies may supersede the recommendations for PET imaging in this section.

† A variety of tumors can occur in the pediatric kidney, and include the following:

® Wilms Tumor
¡ Favorable Histology (FHWT)
¡ Focal Anaplasia (FAWT)
¡ Diffuse Anaplasia (DAWT)
¡ Bilateral Wilms Tumor (BWT)
® Renal Cell Carcinoma (RCC)
® Clear Cell Sarcoma of the Kidney (CCSK)
® Malignant Rhabdoid Tumor of the Kidney (MRT)
® Congenital Mesoblastic Nephroma (CMN)
® Other cancers occurring in the kidney:
¡ Neuroblastoma
¡ Primitive Neuroectodermal Tumor
¡ Rhabdomyosarcoma
¡ Non-Rhabdomyosarcoma Soft Tissue Sarcomas
¡ These and other rare tumors have been reported occurring primarily in the kidney and should be imaged according to the guidelines for the specific histologic diagnosis.

For this condition imaging is medically necessary based on the following criteria:

Unilateral Wilms Tumor Initial Staging:

Many members will present with an asymptomatic abdominal mass, and will undergo ultrasound as a primary evaluation. Doppler ultrasound to evaluate for tumor thrombus is no longer necessary unless CT findings are inconclusive, and should not be performed if CT is already completed.

† CT Abdomen/Pelvis with contrast (CPT^® 74177) is indicated for all unilateral Wilms tumor members

® If bilateral renal lesions are noted on ultrasound or CT, MRI Abdomen (CPT^® 74183) and Pelvis (CPT^® 72197) without and with contrast should be strongly considered for better characterization

† MRI Brain without and with contrast (CPT^® 70553) is indicated for initial staging for any member with neurologic signs or symptoms raising suspicion of CNS metastases as only ~0.5% of Wilms tumor members will ever develop brain metastases

† Bone scan (See PEDONC-1.3) is indicated for any member with signs or symptoms raising suspicion of bony metastases

† PET is not indicated in the initial staging of any pediatric renal tumor

Unilateral Wilms Tumor Treatment Response:

A very low risk subset of stage I FHWT will be observed after nephrectomy, and enter directly into surveillance.

The majority of members will receive chemotherapy with or without XRT, beginning within 14 days of initial surgery.

† CT Chest with (CPT^® 71260) or without contrast (CPT^® 71250) can be performed every 2 cycles during treatment and at the end of planned therapy

† CT Abdomen/Pelvis with contrast (CPT^® 74177) or MRI Abdomen (CPT^® 74183) and Pelvis (CPT^® 72197) without and with contrast can be performed every 2 cycles during treatment and at the end of planned therapy

† PET is not routinely utilized to assess treatment response in Wilms tumor.

® However, since most Wilms tumors are FDG-avid, rare circumstances may occur where PET imaging should be approved to establish the presence of active disease only when a major therapeutic decision depends on PET avidity. These requests will be forwarded for Medical Director review.

† Very low risk FHWT treated with nephrectomy only:

® CT Chest with (CPT^® 71260) or without contrast (CPT^® 71250) OR CXR at 3, 6, 12, and 18 months after nephrectomy
® CT Abdomen and Pelvis with contrast (CPT^® 74177) OR Ultrasound (CPT^® 76700 and 76506) of Abdomen and Pelvis at 3, 6, 12, and 18 months after nephrectomy
® Surveillance pelvic imaging is indicated in this member group due to higher risk of recurrence in surgery only treatment
® Other surveillance imaging should be by Abdominal US (CPT^® 76700) and CXR

® CT Chest with (CPT^® 71260) or without contrast (CPT^® 71250)every 6 months for 3 years after completion of all therapy
® CT Abdomen with contrast (CPT^® 74160), MRI Abdomen without and with contrast (CPT^® 74183), or Ultrasound (CPT^® 76700) of the Abdomen every 6 months for 3 years after completion of all therapy
® Pelvic imaging is not indicated for surveillance unless prior pelvic involvement has been documented or there was tumor rupture at diagnosis
® Other surveillance imaging should be by Abdominal US and CXR

® CT Chest with (CPT^® 71260) or without contrast (CPT^® 71250) every 3 months for 2 years after completion of all therapy
® CT Abdomen and Pelvis with contrast (CPT^® 74177) or MRI Abdomen and Pelvis without and with contrast (CPT^® 74183 and CPT^® 72197) every 3 months for 2 years after completion of all therapy
® Other surveillance imaging should be by Abdominal US and CXR

® Surveillance imaging later than 12 months after completing therapy for recurrence should follow the standard timing listed in this surveillance section.

For this condition imaging is medically necessary based on the following criteria:

Bilateral Wilms Tumor Initial Staging:

Many members will present with an asymptomatic abdominal mass, and will undergo ultrasound as a primary evaluation. Doppler ultrasound to evaluate for tumor thrombus is no longer necessary unless CT findings are inconclusive, and should not be performed if CT is already completed.

Members with bilateral Wilms Tumor may begin therapy without a histologic diagnosis to preserve a localized disease stage and attempt to shrink the tumors to allow for renal-sparing surgical approaches.

† MRI Abdomen and Pelvis without and with contrast (CPT^® 74183 and CPT^® 72197) is the preferred imaging modality for members with bilateral Wilms tumor

® CT Abdomen and Pelvis with contrast (CPT^® 74177) is often performed prior to discovery of bilateral lesions and should not prevent MRI from being approved
® CT Abdomen and Pelvis with contrast (CPT^® 74177) may be used for members with a contraindication to MRI
¡ Avoidance of anesthesia exposure is not a contraindication to MRI for these members

† MRI Brain without and with contrast (CPT^® 70553) is indicated for initial staging for any member with neurologic signs or symptoms raising suspicion of CNS metastases as only ~0.5% of Wilms tumor members will ever develop brain metastases

† Bone scan (See PEDONC-1.3: Modality General Considerations) is indicated for any member with signs or symptoms raising suspicion of bony metastases

† PET is not indicated in the initial staging of any pediatric renal tumor

Bilateral Wilms Tumor Treatment Response:

† MRI Abdomen and Pelvis without and with contrast (CPT^® 74183 and CPT^® 72197) can be performed every 2 cycles during treatment and at the end of planned therapy

® CT Abdomen and Pelvis with contrast (CPT^® 74177) may be used for members with a contraindication to MRI
® If treating with chemotherapy without a biopsy, disease evaluation is indicated at week 6. If either tumor has not shrunk 50%, then open biopsy is indicated to confirm favorable histology.
® If partial nephrectomy still not feasible at week 6, the next disease evaluation is at week 12. Surgical resection should occur no later than week 12.

† PET is not routinely utilized to assess treatment response in Wilms tumor.

® However, since most Wilms tumors are FDG-avid, rare circumstances may occur where PET should be approved to establish the presence of active disease only when a major therapeutic decision depends on PET avidity. These requests will be forwarded for Medical Director review.

† CT Chest with (CPT^® 71260) or without contrast (CPT^® 71250) every 6 months for 3 years after completion of all therapy

† CT Abdomen with contrast (CPT^® 74160) or MRI Abdomen without and with contrast (CPT^®74183) every 6 months for 3 years after completion of therapy

® “Extra” one-time imaging is supported at 3 months after completion of all therapy because close surgical margins occur frequently in members undergoing nephron-sparing surgical approaches, and the risk for early local recurrence is higher

† Other surveillance imaging should be by Abdominal US (CPT^® 76700) and CXR

® When CT or MRI Abdomen no longer indicated, members with bilateral Wilms tumor should have screening Abdominal ultrasound every 3 months until age 8

® Surveillance imaging later than 12 months after completing therapy for recurrence should follow the standard timing listed in this surveillance section.

For this condition imaging is medically necessary based on the following criteria:

A majority of pediatric cases have a novel subtype involving TFE3 or TFEB translocations, which have a different natural history than “adult type” RCC. Members of any age with TFE3 or TFEB translocated RCC should be imaged according to this guideline section.

40 to 45% of pediatric RCC cases have similar histologies to adult RCC (clear cell, papillary, chromophobe, etc.) and imaging decisions will be similar to adult oncology guidelines. Members with all other subtypes of RCC should be imaged according to Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-17: Renal Cell Cancer (RCC).

Pediatric Renal Cell Carcinoma Initial Staging:

Many members will present with an asymptomatic abdominal mass, and will undergo ultrasound as a primary evaluation. Doppler ultrasound to evaluate for tumor thrombus is no longer necessary unless CT findings are inconclusive, and should not be performed if CT is already completed.

† CT Abdomen and Pelvis with contrast (CPT^® 74177) is indicated in all members

® If bilateral renal lesions are noted on ultrasound or CT, MRI Abdomen and Pelvis without and with contrast (CPT^® 74183 and CPT^® 72197) should be strongly considered

† Other staging imaging should be deferred until a histologic diagnosis is made, by complete nephrectomy for most unilateral renal tumors and biopsy for bilateral renal tumors or inoperable unilateral tumors

† MRI Brain without and with contrast (CPT^® 70553) is indicated for any member with neurologic signs or symptoms raising suspicion of CNS metastases

† Bone scan (See PEDONC-1.3: Modality General Considerations) is indicated for any member with signs or symptoms raising suspicion of bony metastases

† PET scan is not indicated in the initial staging of any pediatric renal tumor

Pediatric Renal Cell Carcinoma Treatment Response:

Most members will have surgical resection of all disease at the time of diagnosis and will enter directly into surveillance.

† Members with residual measurable disease after initial surgery and receiving adjuvant medical therapy can have CT Chest with (CPT^® 71260) or without contrast (CPT^® 71250) and CT Abdomen with contrast (CPT^® 74160) every 2 cycles during active treatment

† Pelvic imaging is not indicated unless prior pelvic involvement has been documented

† PET is not routinely utilized to assess treatment response in pediatric RCC.

® However, since some RCC tumors are FDG-avid, rare circumstances may occur where PET should be approved to establish the presence of active disease only when a major therapeutic decision depends on PET avidity these requests will be forwarded for Medical Director review.

† All pediatric RCC members:

® MRI Brain without and with contrast (CPT^® 70553) every 6 months for 2 years after completion of all therapy only for members with documented CNS metastases or new signs/symptoms suggestive of CNS recurrence.

® CT Chest with (CPT^® 71260) or without contrast (CPT^® 71250) every 3 months for 2 years, then every 6 months for 2 years after completion of all therapy
® CT Abdomen with contrast (CPT^® 74160) or MRI Abdomen without and with contrast (CPT^® 74183) every 3 months for 2 years, then every 6 months for 2 years after completion of all therapy
® Pelvic imaging is not indicated for surveillance unless prior pelvic involvement has been documented

® Surveillance imaging is appropriate as listed in the adult Oncology Imaging Guidelines: Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-17.4: Renal Cell Cancer (RCC) – Surveillance

For this condition imaging is medically necessary based on the following criteria:

Be careful not to confuse the diagnosis with clear cell RCC. See Adult Oncology Imaging Policy (Policy #155 in the Radiology Section); ONC-17: Renal Cell Cancer (RCC) for imaging guidelines.

Clear Cell Sarcoma Of The Kidney Initial Staging:

Many members will present with an asymptomatic abdominal mass, and will undergo ultrasound as a primary evaluation. Doppler ultrasound to evaluate for tumor thrombus is no longer necessary unless CT findings are inconclusive, and should not be performed if CT is already completed.

† CT Abdomen and Pelvis with contrast (CPT^® 74177) is indicated in all members

® If bilateral renal lesions are noted on ultrasound or CT, MRI Abdomen and Pelvis without and with contrast should be strongly considered

† Other staging imaging should be deferred until a histologic diagnosis is made, by complete nephrectomy for most unilateral renal tumors and biopsy for bilateral renal tumors or inoperable unilateral tumors

† MRI Brain without and with contrast (CPT^® 70553) is indicated for initial staging in all members with clear cell sarcoma of the kidney

† Bone scan (See PEDONC-1.3: Modality General Considerations) is indicated in all members with clear cell sarcoma of the kidney

† PET is not indicated in the initial staging of any pediatric renal tumor

Clear Cell Sarcoma Of The Kidney Treatment Response:

† CT Chest with (CPT^® 71260) or without contrast (CPT^® 71250) can be performed every 2 cycles during treatment and at the end of planned therapy

† CT Abdomen and Pelvis with contrast (CPT^® 4177) or MRI Abdomen and Pelvis without and with contrast (CPT^® 74183 and CPT^® 72197) can be performed every 6 weeks during treatment and at the end of planned therapy

† MRI Brain without and with contrast (CPT^® 70553) can be performed:

® Every 2 cycles during treatment for members with CNS metastases at initial staging
® At the end of planned therapy for all members with CCSK

† PET is not routinely utilized to assess treatment response in CCSK

® However, since clear cell sarcomas have been shown to be FDG-avid in other anatomic locations, rare circumstances may occur where PET should be approved to establish the presence of active disease only when a major therapeutic decision depends on PET avidity. These requests will be forwarded for Medical Director review.

† CT Chest with (CPT^® 71260) or without contrast (CPT^® 71250) every 3 months for 2 years after completion of all therapy

† CT Abdomen and Pelvis with contrast (CPT^® 74177) or MRI Abdomen and Pelvis without and with contrast (CPT^® 74183 and CPT^® 72197) every 3 months for 2 years after completion of all therapy

† MRI Brain without and with contrast (CPT^® 70553) every 6 months for 3 years after completion of all therapy

† Bone scan (See PEDONC-1.3: Modality General Considerations) every 3 months for 1 year, then every 6 months for 1 year after completion of all therapy

® If negative at 36 months, no further advanced imaging is necessary.

PEDONC-7.6: Malignant Rhabdoid Tumor of the Kidney (MRT) and Other Extracranial Sites

For this condition imaging is medically necessary based on the following criteria:

Be careful not to confuse the diagnosis with rhabdomyosarcoma. See PEDONC-8.2: Rhabdomyosarcoma (RMS) for Imaging Guidelines.

A highly aggressive histologic variant that can also occur in other locations and all non-CNS sites should follow these guidelines.

Primary CNS rhabdoid malignancies should be imaged according to PEDONC-4.5: Atypical Teratoid/Rhabdoid Tumors (ATRT).

Malignant Rhabdoid Tumor Initial Staging:

Many members will present with an asymptomatic abdominal mass, and will undergo ultrasound as a primary evaluation. Doppler ultrasound to evaluate for tumor thrombus is no longer necessary unless CT findings are inconclusive, and should not be performed if CT is already completed.

† CT Abdomen and Pelvis with contrast (CPT^® 74177) is indicated in all members

® If bilateral renal lesions are noted on ultrasound or CT, MRI Abdomen and Pelvis without and with contrast should be strongly considered

† Other staging imaging should be deferred until a histologic diagnosis is made, by complete nephrectomy for most unilateral renal tumors and biopsy for bilateral renal tumors or inoperable unilateral tumors

† MRI Brain without and with contrast (CPT^® 70553) is indicated for all members with MRT of the kidney or other non-CNS site

† Bone scan (See PEDONC-1.3: Modality General Considerations) is indicated in all members with MRT of the kidney or other non-CNS site

† PET is not indicated in the initial staging of any pediatric renal tumor

Malignant Rhabdoid Tumor Treatment Response:

† CT Chest with (CPT^® 71260) or without contrast (CPT^® 71250) can be performed every 2 cycles during treatment and at the end of planned therapy

† CT Abdomen and Pelvis with contrast (CPT^® 74177) or MRI Abdomen and Pelvis without and with contrast (CPT^® 74183 and CPT^® 72197) can be performed every 2 cycles during treatment and at the end of planned therapy

® If primary site other than kidney, perform CT with contrast or MRI without and with contrast of primary site in place of abdominal and pelvic imaging

® Every 2 cycles during treatment for members with CNS metastases at initial staging
® At the end of planned therapy for all members with MRT

† PET is not routinely utilized to assess treatment response in MRT.

® However, since malignant rhabdoid tumors have been shown to be FDG-avid, rare circumstances may occur where PET should be approved to establish the presence of active disease only when a major therapeutic decision depends on PET avidity. These requests will be forwarded for Medical Director review.

† CT Chest with (CPT^® 71260) or without contrast (CPT^® 71250) every 3 months for 2 years after completion of all therapy

† CT Abdomen and Pelvis with contrast (CPT^® 74177) or MRI Abdomen and Pelvis without and with contrast (CPT^® 74183 and CPT^® 72197) every 3 months for 3 years after completion of all therapy

® If primary site other than kidney, perform CT with contrast or MRI without and with contrast of primary site in place of abdominal imaging

† Bone scan (See PEDONC-1.3: Modality General Considerations) every 3 months for 1 year, then every 6 months for 1 year after completion of all therapy only if positive at initial diagnosis

® If negative at 36 months, no further advanced imaging is necessary

PEDONC-7.7: Congenital Mesoblastic Nephroma (CMN)

For this condition imaging is medically necessary based on the following criteria:

This is the most common primary renal tumor occurring in young infants, and the overall prognosis is very good.

Complete surgical removal is curative in most cases, and histologically confirmed metastatic disease or bilateral disease has never been reported.

Congenital Mesoblastic Nephroma Initial Staging

Many members will present with an asymptomatic abdominal mass at the time of birth or abnormal prenatal ultrasound, and will undergo ultrasound as a primary evaluation.

† CT Abdomen and Pelvis with contrast (CPT^® 74177) is indicated in all members

† CT Chest with (CPT^® 71260) can be approved to evaluate inconclusive findings on Chest X-ray

† PET is not indicated in the initial staging of any pediatric renal tumor

Congenital Mesoblastic Nephroma Treatment Response:

† Surgical resection is curative in most members. Children who have resection of the tumor can have a single CT Abdomen and Pelvis with contrast (CPT^® 74177) approved following resection to establish baseline imaging and those with a complete resection should then be imaged according to surveillance guidelines

† Some members will receive preoperative chemotherapy to facilitate safer resection and can have CT Abdomen and Pelvis with contrast (CPT^® 74177) approved every 2 cycles of therapy until surgery, and should then be imaged according to surveillance guidelines after their postoperative baseline imaging study

Congenital Mesoblastic Nephroma Surveillance Imaging

† Recurrences are rare, but most occur within 12 months of diagnosis

† Given the young age of the members with CMN, ultrasound is the preferred surveillance imaging modality to avoid radiation and anesthesia exposures

® CT Abdomen and Pelvis with contrast (CPT^® 74177) or MRI Abdomen and Pelvis without and with contrast (CPT^® 74183 and CPT^® 72197) can be approved every 3 months for 1 year after completion of all therapy for members with residual abnormalities present on post-operative imaging or inconclusive findings on ultrasound

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10. Uslu L, Doing J, Link M, Rosenburg J, Quon A, Daldrup-Link HE. Value of ¹⁸F-FDG PET and PET/CT for Evaluation of Pediatric Malignancies. J Nucl Med. 2015;56(2):274-286. doi:10.2967/jnumed.114.146290.
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12. Venkatramani R, Chi Y-Y, Coppes MJ, et al. Outcome of patients with intracranial relapse enrolled on National Wilms Tumor Study Group clinical trials. Pediatr Blood Cancer. 2017;64(7):e26406. doi:10.1002/pbc.26406.
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14. Ehrlich P, Chi Y-Y, Chintagumpala MM, et al. results of the first prospective multi-institutional treatment study in children with bilateral Wilms tumor (AREN0534): a report from the Children’s Oncology Group. Ann Surg. 2017;266(3):470-478. doi:10.1097/SLA.0000000000002356.
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