Pineal and nonpineal supratentorial primitive neuroectodermal tumors

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 sur...

Full description

Saved in:
Bibliographic Details
Published in:Child's nervous system 1999-10, Vol.15 (10), p.586-591
Main Author: Jakacki, R I
Format: Article
Language:English
Subjects:
Child, Preschool
Combined Modality Therapy
Humans
Infant
Neuroectodermal Tumors, Primitive - drug therapy
Neuroectodermal Tumors, Primitive - therapy
Pineal Gland
Prognosis
Supratentorial Neoplasms - drug therapy
Supratentorial Neoplasms - therapy
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:0256-7040
1433-0350
DOI:10.1007/s003810050547