Treatment strategies for high-risk medulloblastoma and supratentorial primitive neuroectodermal tumors. Review of the literature.
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.
Jakacki
Regina I
RI
Division of Pediatric Hematology/Oncology, Children's Hospital of Pittsburgh, Pennsylvania 15213, USA. regina.jakacki@chp.edu
D000367:Age Factors; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000493:pharmacokinetics / Q000627:therapeutic use; D001812:Blood-Brain Barrier; D001932:Brain Neoplasms / Q000188:drug therapy / Q000503:physiopathology / Q000532:radiotherapy* / Q000601:surgery*; D006801:Humans; D008527:Medulloblastoma / Q000188:drug therapy / Q000503:physiopathology / Q000532:radiotherapy* / Q000601:surgery*; D009422:Nervous System Diseases / Q000209:etiology / Q000517:prevention & control; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000503:physiopathology / Q000532:radiotherapy* / Q000601:surgery*; D011379:Prognosis; D011832:Radiation Injuries; D012307:Risk Factors
D000970:Antineoplastic Agents