title: Radiation is an important component of multimodality therapy for pediatric non-pineal supratentorial primitive neuroectodermal tumors.
abstract: OBJECTIVE
  To review a historical cohort of pediatric patients with supratentorial primitive neuroectodermal tumors (sPNET), to clarify the role of radiation in the treatment of these tumors.
  
  
  METHODS
  Fifteen children aged <18 years with non-pineal sPNETs diagnosed between 1992 and 2006 were identified. Initial therapy consisted of surgical resection and chemotherapy in all patients and up-front radiotherapy (RT) in 5 patients. Five patients had RT at the time of progression, and 5 received no RT whatever. Kaplan-Meier estimates of overall survival were then calculated.
  
  
  RESULTS
  The median follow-up from diagnosis for all patients was 31 months (range, 0.5-165 months) and for surviving patients was 49 months (range, 10-165). Of the 5 patients who received up-front RT, all were alive without evidence of disease at a median follow-up of 50 months (range, 25-165 months). Only 5 of the 10 patients who did not receive up-front RT were alive at last follow-up. There was a statistically significant difference in overall survival between the patient group that received up-front RT and the group that did not (p = 0.048). In addition, we found a trend toward a statistically significant improvement in overall survival for those patients who received gross total resections (p = 0.10).
  
  
  CONCLUSIONS
  Up-front RT and gross total resection may confer a survival benefit in patients with sPNET. Local failure was the dominant pattern of recurrence. Efforts should be made to determine patients most likely to have local failure exclusively or as a first recurrence, in order to delay or eliminate craniospinal irradiation.
authors.affiliation: Department of Radiation Oncology, University of California-San Francisco, San Francisco, CA.
authors.forename: Sean M
authors.initials: SM
authors.lastname: McBride
authors.forename: Sally M
authors.initials: SM
authors.lastname: Daganzo
authors.forename: Anuradha
authors.initials: A
authors.lastname: Banerjee
authors.forename: Nalin
authors.initials: N
authors.lastname: Gupta
authors.forename: Kathleen R
authors.initials: KR
authors.lastname: Lamborn
authors.forename: Michael D
authors.initials: MD
authors.lastname: Prados
authors.forename: Mitchel S
authors.initials: MS
authors.lastname: Berger
authors.forename: William M
authors.initials: WM
authors.lastname: Wara
authors.forename: Daphne A
authors.initials: DA
authors.lastname: Haas-Kogan
mesh_terms: D017668:Age of Onset; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009367:Neoplasm Staging; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery; D010870:Pineal Gland / Q000473:pathology; D011878:Radiotherapy / Q000379:methods; D013116:Spinal Cord / Q000473:pathology; D033581:Stem Cell Transplantation; D016019:Survival Analysis; D017741:Survivors; D013997:Time Factors