title: Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation, and chemotherapy.
abstract: BACKGROUND
  The outcome of a child with a primitive neuroectodermal tumors arising supratentorially (SPNET) is not well characterized and may differ from the outcome of a patient with a histologically similar cerebellar tumor (medulloblastoma [MB]). Recently, 5-year progression free survival rates as high as 80% have been reported for children with MB treated with craniospinal radiation (CRT) and chemotherapy including cisplatin, lomustine (CCNU), and vincristine (VCR).
  
  
  METHODS
  The authors reviewed the outcome of 22 consecutive patients age 3 years and older (mean age, 10 years; range, 3-18 years) with SPNET who were treated at the study institutions between 1981 and 1996. Tumor location included was 13 pineal, 6 cortical, and 3 thalamic or suprasellar. Five patients had disease dissemination at diagnosis. All patients underwent surgery and staging, followed by CRT and chemotherapy with cisplatin, CCNU, and VCR.
  
  
  RESULTS
  Of the 22 patients, 13 had developed disease progression and 10 had died at the time of last follow-up. Overall progression free survival (PFS) was 47% +/- 11% at 3 years and 37% +/- 11% at 5 years. There was a significant difference in PFS between patients with localized disease versus those with disseminated disease (P = 0.04). There was no statistical association between tumor location and survival. Although not significant (P = 0.21), there was a trend toward better survival of those patients with complete or near-complete resection compared with those with partial resection or biopsy.
  
  
  CONCLUSIONS
  The results of the current study demonstrate that the outcome for children with SPNET treated with radiation and chemotherapy appears worse than for children with MB treated with identical therapy. This suggests that there may be biologic differences between supratentorial and infratentorial primitive neuroectodermal tumors, thus requiring refinements in treatment.
authors.affiliation: Department of Pediatrics, Children's Hospital of Alabama, University of Alabama at Birmingham, 35233, USA.
authors.forename: A T
authors.initials: AT
authors.lastname: Reddy
authors.forename: A J
authors.initials: AJ
authors.lastname: Janss
authors.forename: P C
authors.initials: PC
authors.lastname: Phillips
authors.forename: H L
authors.initials: HL
authors.lastname: Weiss
authors.forename: R J
authors.initials: RJ
authors.lastname: Packer
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000008:administration & dosage; D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage; D000972:Antineoplastic Agents, Phytogenic / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002540:Cerebral Cortex / Q000187:drug effects / Q000528:radiation effects / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D016371:Cranial Irradiation; D018450:Disease Progression; D018572:Disease-Free Survival; D005500:Follow-Up Studies; D006801:Humans; D016014:Linear Models; D008130:Lomustine / Q000008:administration & dosage; D009367:Neoplasm Staging; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D010871:Pinealoma / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D012189:Retrospective Studies; D015173:Supratentorial Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D015996:Survival Rate; D013786:Thalamic Diseases / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D000970:Antineoplastic Agents; D018906:Antineoplastic Agents, Alkylating; D000972:Antineoplastic Agents, Phytogenic; D014750:Vincristine; D008130:Lomustine; D002945:Cisplatin