Treatment strategies for high-risk medulloblastoma and supratentorial primitive neuroectodermal tumors. Review of the literature

J Neurosurg. 2005 Jan;102(1 Suppl):44-52. doi: 10.3171/ped.2005.102.1.0044.


Primitive neuroectodermal tumors (PNETs) are malignant tumors with a high propensity to disseminate throughout the cerebrospinal fluid. Current treatment guidelines are largely determined by clinically based prognostic factors, the most important of which are tumor location and the extent of tumor spread. Although the cure rate for high-risk PNETs has improved, the irreversible sequelae of craniospinal axis radiation treatment in patients who survive have motivated researchers to investigate more fully which patients can safely receive less treatment. The author reviews the literature, describes currently available treatment options for patients with high-risk PNETs, and discusses strategies aimed at improving outcome and refining prognosis that are currently being explored.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Antineoplastic Agents / administration & dosage
  • Antineoplastic Agents / pharmacokinetics
  • Antineoplastic Agents / therapeutic use
  • Blood-Brain Barrier
  • Brain Neoplasms / drug therapy
  • Brain Neoplasms / physiopathology
  • Brain Neoplasms / radiotherapy*
  • Brain Neoplasms / surgery*
  • Humans
  • Medulloblastoma / drug therapy
  • Medulloblastoma / physiopathology
  • Medulloblastoma / radiotherapy*
  • Medulloblastoma / surgery*
  • Nervous System Diseases / etiology
  • Nervous System Diseases / prevention & control
  • Neuroectodermal Tumors, Primitive / drug therapy
  • Neuroectodermal Tumors, Primitive / physiopathology
  • Neuroectodermal Tumors, Primitive / radiotherapy*
  • Neuroectodermal Tumors, Primitive / surgery*
  • Prognosis
  • Radiation Injuries*
  • Risk Factors


  • Antineoplastic Agents