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


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.


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.


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.
Johnston
Donna L
DL
Division of Hematology/Oncology, Children's Hospital of Eastern Ontario, 401 Smyth Road, Ottawa, ON, Canada, K1H 8L1. djohnston@cheo.on.ca
Keene
Daniel L
DL
Lafay-Cousin
Lucie
L
Steinbok
Paul
P
Sung
Lillian
L
Carret
Anne-Sophie
AS
Crooks
Bruce
B
Strother
Douglas
D
Wilson
Beverly
B
Odame
Isaac
I
Eisenstat
David D
DD
Mpofu
Chris
C
Zelcer
Shayna
S
Huang
Annie
A
Bouffet
Eric
E
D000293:Adolescent; D001932:Brain Neoplasms / Q000453:epidemiology* / Q000628:therapy; D002170:Canada / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006306:Health Surveys; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D018242:Neuroectodermal Tumors, Primitive / Q000453:epidemiology* / Q000628:therapy; D010372:Pediatrics; D012189:Retrospective Studies; D015173:Supratentorial Neoplasms / Q000453:epidemiology* / Q000628:therapy; D011795:Surveys and Questionnaires; D016019:Survival Analysis