title: Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: a Childrens Cancer Group randomized trial.
abstract: OBJECTIVE
  To determine clinical characteristics and response to treatment for children with supratentorial primitive neuroectodermal tumors (S-PNETs).
  
  
  METHODS
  After surgery and staging, 55 patients aged 1.5 to 19.3 years with S-PNETs were randomized to receive craniospinal radiotherapy (RT) followed by eight cycles of 1-(2-chloro-ethyl)-3-cyclohexylnitrosourea (CCNU), vincristine (VCR), and prednisone (standard treatment) or two cycles of 8-in-1 chemotherapy followed by RT and then eight additional cycles of 8-in-1.
  
  
  RESULTS
  Three-year Kaplan-Meier estimates (estimate +/- SE) of survival and progression-free survival (PFS) rates for patients with confirmed diagnoses of S-PNET were 57% +/- 8% and 45% +/- 8%, respectively; survival and PFS rates for children with PNETs located in the pineal region were 73% +/- 12% and 61% +/- 13%, respectively, and were significantly different from the other S-PNETs (P < .03). The 8-in-1 arm had greater toxicity than the standard-treatment arm. Distributions of PFS between the two treatment groups were not significantly different (P > .5). Other univariate prognostic factors that influenced PFS included metastasis (M) stage (P < .03: M0 50% +/- 9% v M1-4 0%) and age (P < .02: 1.5 to 2 years 25% +/- 13% v > or = 3 years 53% +/- 9%).
  
  
  CONCLUSIONS
  In this first randomized treatment trial for S-PNETs in children, no significant differences were detected between the two treatment groups. M0 and pineal site of involvement were independent predictors of a better outcome. However, survival was better than previously reported.
authors.affiliation: Cleveland Clinic Foundation, OH, USA.
authors.forename: B H
authors.initials: BH
authors.lastname: Cohen
authors.forename: P M
authors.initials: PM
authors.lastname: Zeltzer
authors.forename: J M
authors.initials: JM
authors.lastname: Boyett
authors.forename: J R
authors.initials: JR
authors.lastname: Geyer
authors.forename: J C
authors.initials: JC
authors.lastname: Allen
authors.forename: J L
authors.initials: JL
authors.lastname: Finlay
authors.forename: P
authors.initials: P
authors.lastname: McGuire-Cullen
authors.forename: J M
authors.initials: JM
authors.lastname: Milstein
authors.forename: L B
authors.initials: LB
authors.lastname: Rorke
authors.forename: P
authors.initials: P
authors.lastname: Stanley
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D008130:Lomustine / Q000008:administration & dosage; D008297:Male; D009367:Neoplasm Staging; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D010870:Pineal Gland; D010871:Pinealoma / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D011241:Prednisone / Q000008:administration & dosage; D011379:Prognosis; D011446:Prospective Studies; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D008130:Lomustine; D011241:Prednisone