Adult neuroectodermal tumors of posterior fossa (medulloblastoma) and of supratentorial sites (stPNET)

Alba A. Brandes, Enrico Franceschi, Alicia Tosoni, Michele Reni, Gemma Gatta, Charles Vecht, Rolf D. Kortmann

Research output: Contribution to journalArticlepeer-review

Abstract

Medulloblastoma and supratentorial primitive neuroectodermal tumors are rare diseases in adults. Due to this rarity, few prospective clinical trials have been conducted on medulloblastoma in adults, investigations being based exclusively on retrospective studies; the populations considered in literature are small, and the different treatments given span decades, during which diagnostic procedures, neurosurgical skills and radiotherapy techniques have changed. Unlike pediatric patients, adult medulloblastoma patients have been treated according to risk-adapted therapeutic strategies in only a few series and despite risk-tailored treatments, 20-30% of patients experience recurrence. Although patients could respond to second line treatments, the prognosis of relapsed patients remains dismal. An important challenge for the future will be the biological characterization of medulloblastoma, with the identification of specific genetic patterns of patients with a better or a worse prognosis.

Original languageEnglish
Pages (from-to)165-179
Number of pages15
JournalCritical Reviews in Oncology/Hematology
Volume71
Issue number2
DOIs
Publication statusPublished - Aug 2009

Keywords

  • Chemotherapy
  • Long-term survival
  • Medulloblastoma
  • Radiotherapy
  • Supratentorial PNET
  • Surgery

ASJC Scopus subject areas

  • Oncology
  • Hematology
  • Geriatrics and Gerontology

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