Brain tumors are the second most common group of malignancies in children.
For some pediatric tumors such as medulloblastoma, supratentorial primitive neuroectodermal tumor (PNET), ependymoma and germ cell tumors, leptomeningeal dissemination is a route of spread. Imaging of the entire neuraxis is performed in addition to cerebral spinal fluid (CSF) cytology from a lumbar tap.
For low-grade gliomas, surgery is the mainstay of treatment. Adjuvant therapy is not needed for patients who have gross total resections.
Diffuse pontine gliomas are the most common brainstem gliomas in children. Radiation to a dose of 54 Gy in 30 fractions over 6 weeks is the standard treatment.
Current treatment strategies in medulloblastoma, which include maximal safe resection, craniospinal irradiation (CSI) followed by primary site irradiation, and chemotherapy, have resulted in 5-year progression-free survivals of 80 and 70% for standard- and high-risk patients.
The extent of resection is the most important prognostic factor for ependymoma.
Adjuvant radiotherapy is currently recommended for ependymoma, regardless of extent of resection.
Germ cell tumors are divided into germinoma and nongerminomatous germ cell tumor (NGGCT), with the former having a better prognosis. Germinomas are highly curable with radiotherapy alone, while NGGCT is best managed with a combination of radiation and chemotherapy.
Late effects of radiotherapy in young children (<3 years of age) with medulloblastoma, ependymoma, and malignant glioma have prompted investigators to delay or possibly eliminate radiotherapy by using chemotherapy.
The current trend for treatment of craniopharyngiomas is limited surgery or biopsy followed by radiotherapy, as achieving a gross total resection may result in significant morbidity because of the proximity of critical structures such as the optic chiasm and nerves, circle of Willis, and hypothalamus to the tumor.