Medulloblastoma: clinical and biologic aspects

Neuro Oncol. 1999 Jul;1(3):232-50. doi: 10.1093/neuonc/1.3.232.


Medulloblastoma is the most common childhood primary CNS tumor, and treatment approaches have evolved over the past three decades. The biologic underpinnings of medulloblastoma are not fully characterized, but recent work has identified new, important directions for research. Stratification of patients with medulloblastoma into risk groups is the backbone of most ongoing therapeutic studies. Patients are usually characterized as being either average risk or poor risk, although an intermediate risk group may exist. Standard treatment for older children with medulloblastoma consists of radiation and, for most, chemotherapy. Children with nondisseminated disease at the time of diagnosis have been reported to have as high as an 80% five-year disease-free survival rate after treatment with reduced dose (2340 cGy) craniospinal irradiation, local boost radiation therapy (5500 cGy), and chemotherapy, given during and after radiation therapy. Preradiation chemotherapy has yet to be shown to be of benefit for children with medulloblastoma. Children with disseminated disease are a highly problematic subgroup of patients to treat. A variety of new approaches are being studied, most of which are intensifying chemotherapy either prior to or after radiation. Long-term survivors of medulloblastoma are at significant risk for permanent endocrinologic, cognitive, and psychological sequelae. Infants and very young children with medulloblastoma remain a difficult therapeutic challenge because they have the most virulent form of the disease and are at highest risk for treatment-related sequelae.

Publication types

  • Review

MeSH terms

  • Adult
  • Age Factors
  • Animals
  • Antineoplastic Combined Chemotherapy Protocols / adverse effects
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Biomarkers, Tumor / analysis
  • Brain Damage, Chronic / etiology
  • Cell Differentiation
  • Cerebellar Neoplasms / diagnosis
  • Cerebellar Neoplasms / drug therapy
  • Cerebellar Neoplasms / epidemiology
  • Cerebellar Neoplasms / pathology*
  • Cerebellar Neoplasms / radiotherapy
  • Cerebellar Neoplasms / surgery
  • Chemotherapy, Adjuvant
  • Child
  • Child, Preschool
  • Cognition Disorders / etiology
  • Combined Modality Therapy
  • Cranial Fossa, Posterior
  • Cranial Irradiation / adverse effects
  • Diagnostic Imaging
  • Diseases in Twins
  • Forecasting
  • Growth Disorders / etiology
  • Humans
  • Infant
  • Infant, Newborn
  • Medulloblastoma / diagnosis
  • Medulloblastoma / drug therapy
  • Medulloblastoma / epidemiology
  • Medulloblastoma / pathology*
  • Medulloblastoma / radiotherapy
  • Medulloblastoma / surgery
  • Middle Aged
  • Neoplasm Proteins / analysis
  • Neoplasm Recurrence, Local
  • Neuroectodermal Tumors, Primitive / classification
  • Neuroectodermal Tumors, Primitive / drug therapy
  • Neuroectodermal Tumors, Primitive / epidemiology
  • Neuroectodermal Tumors, Primitive / pathology
  • Neuroectodermal Tumors, Primitive / radiotherapy
  • Radiation Injuries / etiology
  • Radiation Tolerance
  • Radiotherapy, Adjuvant
  • Rats
  • Risk Factors


  • Biomarkers, Tumor
  • Neoplasm Proteins