title: Non-pineal supratentorial primitive neuro-ectodermal tumors (sPNET) in teenagers and young adults: Time to reconsider cisplatin based chemotherapy after cranio-spinal irradiation?
abstract: BACKGROUND
  Supratentorial PNET (sPNET) are rare CNS tumors of embryonal origin arising in children and adults. The treatment of sPNET for all age groups at our cancer center has been based on the management of medulloblastoma (MB), involving neurosurgical debulking followed by cranio-spinal irradiation (CSI) and systemic chemotherapy.
  
  
  METHODS
  Medical records were reviewed to gather demographic and clinical data about all embryonal CNS tumors in children and adults from 2001 to 2007. Tumor pathology, clinical management and survival data were also assessed, particularly as regards those patients who received the Packer chemotherapy regimen for either sPNET or MB.
  
  
  RESULTS
  Eleven patients (five children and six adults) were identified with non-pineal sPNET, three children with pineal sPNET, and 19 patients (18 children and 1 adult) with MB. There was no difference in overall survival (OS) rates between pediatric and adult sPNET. When all sPNET were compared to all MB, 5-year OS was 14% versus 73%, respectively, but was only 9% for non-pineal sPNET. When only considering those patients treated with the Packer chemotherapy regimen, the 5-year OS was 12% for sPNET versus 79% for MB.
  
  
  CONCLUSIONS
  This retrospective study demonstrates that non-pineal sPNET are clinically distinct from MB and are resistant to the Packer chemotherapy regimen. We suggest that it is time to reconsider the use of this regimen in teenage and young adult non-pineal sPNET and to investigate the utility of alternative approaches.
authors.affiliation: Division of Oncology, Oncology Centre, Addenbrooke's Hospital, Cambridge, United Kingdom.
authors.forename: Swethajit
authors.initials: S
authors.lastname: Biswas
authors.forename: Amos
authors.initials: A
authors.lastname: Burke
authors.forename: Sheen
authors.initials: S
authors.lastname: Cherian
authors.forename: Denise
authors.initials: D
authors.lastname: Williams
authors.forename: James
authors.initials: J
authors.lastname: Nicholson
authors.forename: Gail
authors.initials: G
authors.lastname: Horan
authors.forename: Sarah
authors.initials: S
authors.lastname: Jefferies
authors.forename: Michael
authors.initials: M
authors.lastname: Williams
authors.forename: Helena M
authors.initials: HM
authors.lastname: Earl
authors.forename: Neil G
authors.initials: NG
authors.lastname: Burnet
authors.forename: Helen
authors.initials: H
authors.lastname: Hatcher
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002528:Cerebellar Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D002648:Child; D002945:Cisplatin / Q000008:administration & dosage; D016371:Cranial Irradiation; D005500:Follow-Up Studies; D006801:Humans; D008130:Lomustine / Q000008:administration & dosage; D008527:Medulloblastoma / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D009367:Neoplasm Staging; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D012189:Retrospective Studies; D015173:Supratentorial Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D015996:Survival Rate; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage; D055815:Young Adult
chemical_list: D014750:Vincristine; D008130:Lomustine; D002945:Cisplatin