NCCN Releases New Guidelines for Pediatric CNS Cancers

The National Comprehensive Cancer Network (NCCN) issued recommendations for providers treating children with brain cancers, the second-most-common pediatric malignancy and the leading cause of cancer-related death in children.^1-3

Approximately 15% of all intracranial neoplasms appear in patients younger than 19 years. The 5-year overall survival rate is less than 20%.

Anita Mahajan, MD, a radiation oncologist with Mayo Clinic Rochester and vice-chair of the NCCN Guidelines Panel for Pediatric CNS Cancers, said in a press release that the new guidelines provide evidence-based recommendations across treatment disciplines, including oncologists/neuro-oncologists, radiation oncologists, pathologists, and pediatric neurosurgeons. The guidelines recommend that all patients with high-grade gliomas should receive care from “a multidisciplinary team with experience managing central nervous system [CNS] tumors.”⁴

Treatment for pediatric CNS tumors often includes a combination of surgery, radiation therapy, and chemotherapy. The NCCN also recommends evaluating the patient for genetic counseling and/or evaluation for cancer predisposition.

“Treating children with cancer is very different from adults, particularly for CNS tumors,” Amar Gajjar, MD, the chair of the Pediatric CNS Cancers Panel and chair of the Department of Pediatrics at St. Jude Children’s Research Hospital, stated in a press release. “We need to be extra careful to not impact physical and cognitive development and to protect against long term [adverse] effects [AEs].”

The most common symptoms of CNS cancers include headache, nausea, and vomiting, which are often the result of increased intracranial pressure. Other presenting symptoms can include seizure, hemiparesis, monoparesis, cranial nerve deficits, ataxia, hemisensory loss, dysphasia, aphasia, and memory impairment. Infants may present with increased head circumference and loss of developmental milestones.

Inherited risk factors include neurofibromatosis type 1, Li-Fraumeni syndrome, Turcot syndrome, Lynch syndrome, constitutional mismatch repair deficiency, and exposure to ionizing radiation. Previous data has shown that external beam radiation therapy is associated with subsequent CNS malignancies.⁵ Such tumors are a particular concern in pediatric patients because of their long life expectancies.

“Radiation is a key component when treating adults, but options to avoid or reduce radiation in young children are important, especially those under age 3,” Gajjar added. “These guidelines provide a snapshot of how leading experts from across the country are applying the current evidence to recommend the best approach for infants, young children, and adolescents into early adulthood. We’re committed to update these guidelines at least once a year moving forward.”

The NCCN has now released 5 guidelines focusing on children’s cancers following publication of expert consensus guidelines for pediatric acute lymphoblastic leukemia, pediatric aggressive mature B-cell lymphomas, pediatric Hodgkin lymphoma, and Wilms tumors. The network intends to issue recommendations covering at least 90% of all incident childhood cancers. NCCN currently provides comprehensive management recommendations for 97% of all incident cancer in the United States and globally, plus screening, prevention, and supportive care including survivorship best practices.

“We’re planning to expand the guidelines to include other CNS tumor types,” Mahajan said. “Right now, the science is advancing as we learn more about how to differentiate and characterize these tumors. In the future, with more trial results coming in, we anticipate an increase in personalized medicine with treatments targeted to the specific patient and tumor.”

References

1. National Comprehensive Cancer Network shares new recommendations for treating children with brain tumors. NCCN. News release. July 12, 2022. Accessed July 13, 2022. https://prn.to/3RxZqsc
2. Pfister SM, Reyes-Múgica M, Chan JKC, et al. A summary of the inaugural who classification of pediatric tumors: transitioning from the optical into the molecular era. Cancer Discov. 2022;12(2):331-355. doi: 10.1158/2159-8290.CD-21-1094
3. Siegel RL, Miller KD, Fuchs HE, Jemal A. Cancer statistics, 2022. CA Cancer J Clin. 2022;72(1):7-33. doi: 10.3322/caac.21708
4. Gajjar A, Mahajan A, Abdelbaki M, et al. Pediatric central nervous system cancers: version 1.2023. July 12, 2022. Accessed July 13, 2022. https://bit.ly/3O96LLY
5. Tsui K, Gajjar A, Li C, Srivastava D, et al. Subsequent neoplasms in survivors of childhood central nervous system tumors: risk after modern multimodal therapy. Neuro Oncol. 2015;17(3):448-56. doi: 10.1093/neuonc/nou279