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Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report.

Authors :
Johnston DL
Keene DL
Lafay-Cousin L
Steinbok P
Sung L
Carret AS
Crooks B
Strother D
Wilson B
Odame I
Eisenstat DD
Mpofu C
Zelcer S
Huang A
Bouffet E
Source :
Journal of neuro-oncology [J Neurooncol] 2008 Jan; Vol. 86 (1), pp. 101-8. Date of Electronic Publication: 2007 Jul 10.
Publication Year :
2008

Abstract

Introduction: Supratentorial primitive neuroectodermal tumors (SPNET) are rare tumors accounting for only 2.5% of childhood brain tumors. The purpose of this study was to describe the range of treatment regimens used to treat pediatric SPNET in Canada and to identify prognostic factors for overall survival in this population.<br />Methods: This study was a retrospective clinical analysis of SPNET patients treated over the last 10 years in Canada. A questionnaire was developed and distributed to all institutions in Canada who treat pediatric patients. Data were collected for patients <19 years of age who were diagnosed and treated for SPNET between 1995 and 2005.<br />Results: Data were obtained for 48 eligible patients. The stages of patients for whom complete data were provided were 80, 3, and 16% for metastatic stage M0, M1, and M2/3, respectively. The best responses to therapy included complete response in 44%, partial response in 8%, still on therapy in 2%, progressive disease in 31%, toxic death in 2%, and no therapy given in 12%. The 4-year survival was 37.7 +/- 7.6%. The factors associated with an increase in survival were the use of radiation therapy and chemotherapy, and age >2 years. Overall survival was not affected by metastatic disease at diagnosis, tumor site, or degree of initial resection.<br />Conclusions: Survival is poor in SPNET patients but highest in those who received chemotherapy and radiation therapy. Further studies are needed to improve the survival of these patients.

Details

Language :
English
ISSN :
0167-594X
Volume :
86
Issue :
1
Database :
MEDLINE
Journal :
Journal of neuro-oncology
Publication Type :
Academic Journal
Accession number :
17619825
Full Text :
https://doi.org/10.1007/s11060-007-9440-1