Child's Nervous System

, Volume 15, Issue 10, pp 586–591

Pineal and nonpineal supratentorial primitive neuroectodermal tumors

  • Regina I. Jakacki
State of the Art

DOI: 10.1007/s003810050547

Cite this article as:
Jakacki, R. Child's Nerv Syst (1999) 15: 586. doi:10.1007/s003810050547

Abstract 

Pineal region supratentorial primitive neuroectodermal tumors (SPNETs; pineoblastomas) and nonpineal SPNETs are rare tumors that historically have carried a very poor prognosis. With multimodality therapy, including maximal surgical resection, craniospinal radiation therapy and chemotherapy, the survival for patients with pineal PNETs has significantly improved. Chemotherapy alone, at least in conventional doses, appears to be insufficient treatment for younger children with pineoblastomas, in whom there is almost universal rapid tumor progression and death. Survival of patients with nonpineal SPNETs remains in the order of 30–35% despite multimodality therapy. Unlike those with pineal SPNETs, a significant percentage of infants with nonpineal SPNETs who undergo gross total surgical resection followed by chemotherapy will be long-term survivors. This article gives an overview of the natural history, prognostic factors and treatment of both pineal and nonpineal SPNETs.

Key words Supratentorial primitive neuroectodermal tumorSPNETPineoblastomaTreatmentPrognosis

Copyright information

© Springer-Verlag Berlin Heidelberg 1999

Authors and Affiliations

  • Regina I. Jakacki
    • 1
  1. 1.Children’s Hospital of Pittsburgh, 3520 Fifth Avenue, Suite 205, Pittsburgh, PA 15213, USA e-mail: jakackr@chplink.chp.edu Tel.: +1-412-692-5056 Fax: +1-412-692-7693US