Risk-Adapted Therapy for Young Children With Embryonal Brain Tumors, Choroid Plexus Carcinoma, High Grade Glioma or Ependymoma

Histologically confirmed newly diagnosed CNS tumors of any of the following :

• Medulloblastoma (all histologic subtypes, including medullomyoblastoma and melanotic medulloblastoma)
• Supratentorial primitive neuroectodermal tumor (PNET) (including CNS neuroblastoma or ganglioneuroblastoma, medulloepithelioma, and ependymoblastoma)
• Pineoblastoma
• Atypical teratoid rhabdoid tumor (ATRT)
• Choroid plexus carcinoma
• High grade glioma (including anaplastic astrocytoma, anaplastic oligodendroglioma, anaplastic ganglioglioma, pleomorphic xanthoastrocytoma with anaplastic features, high-grade astroblastoma , anaplastic pilocytic astrocytoma, malignant glioneuronal tumor, glioblastoma multiforme), or gliosarcoma,
• Ependymoma (including all ependymoma histological variants)

• Age < 3 years at time of diagnosis for all histological diagnosis. Medulloblastoma patients ≥ 3 and < 5years old at diagnosis who have non-metastatic disease with no more than 1cm2 of residual tumor are also eligible.

  • Meets criteria for 1 of the following risk groups:

• Low-risk group:

  • Histologically confirmed nodular desmoplastic medulloblastoma, including medulloblastoma with extensive nodularity

    • Focal areas of anaplasia or other atypical features suggesting more aggressive phenotype in a tumor otherwise considered nodular desmoplastic should be treated on the intermediate-risk group, with final risk stratification at the discretion of principal investigator and study pathologist

  • No evidence of CNS metastasis 7 to 28 days after surgery by MRI and cytologic examination of lumbar cerebrospinal fluid (CSF)

    • Ventricular CSF from a shunt or Ommaya reservoir may be used to rule out M1 disease when lumbar puncture is medically contraindicated
    • Intermediate-risk group assignment when there is no other evidence of metastasis and CSF sampling is not possible
  • Gross total resection, defined as residual tumor or imaging abnormality (not definitive for residual tumor) with a size of < 1 cm2 confirmed on postoperative CT scan or MRI
  • Brain stem invasion by the tumor in the absence of imaging evidence of residual tumor (tumor size < 1 cm2) and otherwise meets criteria for the low-risk group, the patient will be classified as low-risk
  • Desmoplastic medulloblastoma patients who are ≥3 -<5 years of age will NOT be eligible for the low risk arm of the protocol.

• Intermediate-risk group:

  • Histologically confirmed nodular desmoplastic medulloblastoma with less than gross total resection and no evidence of metastasis
  • Any eligible histologic diagnosis other than desmoplastic medulloblastoma with no evidence of CNS metastasis
  • Medulloblastoma patients who are ≥3 and < 5 yrs of age irrespective of histology and with no evidence of CNS metastasis

• High-risk group:

  • Any eligible histologic diagnosis with evidence of CNS metastasis
  • Patients with extraneural metastasis are eligible for treatment on the high-risk group

PATIENT CHARACTERISTICS:

• Lansky performance status ≥ 30 (except for posterior fossa syndrome)
• WBC > 2,000/mm3
• Platelets > 50,000/mm3 (without support)
• Hemoglobin > 8 g/dL (with or without support)
• ANC > 500/mm3
• Serum creatinine < 3 times upper limit of normal (ULN)
• ALT < 5 times ULN
• Total bilirubin < 3 times ULN

PRIOR CONCURRENT THERAPY:

• See Disease Characteristics
• No more than 31 days since prior definitive surgery
• No prior radiotherapy or chemotherapy other than corticosteroid therapy