title: Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report.
abstract: 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.
authors.affiliation: Division of Hematology/Oncology, Children's Hospital of Eastern Ontario, 401 Smyth Road, Ottawa, ON, Canada, K1H 8L1. djohnston@cheo.on.ca
authors.forename: Donna L
authors.initials: DL
authors.lastname: Johnston
authors.forename: Daniel L
authors.initials: DL
authors.lastname: Keene
authors.forename: Lucie
authors.initials: L
authors.lastname: Lafay-Cousin
authors.forename: Paul
authors.initials: P
authors.lastname: Steinbok
authors.forename: Lillian
authors.initials: L
authors.lastname: Sung
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Carret
authors.forename: Bruce
authors.initials: B
authors.lastname: Crooks
authors.forename: Douglas
authors.initials: D
authors.lastname: Strother
authors.forename: Beverly
authors.initials: B
authors.lastname: Wilson
authors.forename: Isaac
authors.initials: I
authors.lastname: Odame
authors.forename: David D
authors.initials: DD
authors.lastname: Eisenstat
authors.forename: Chris
authors.initials: C
authors.lastname: Mpofu
authors.forename: Shayna
authors.initials: S
authors.lastname: Zelcer
authors.forename: Annie
authors.initials: A
authors.lastname: Huang
authors.forename: Eric
authors.initials: E
authors.lastname: Bouffet
mesh_terms: 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