title: Outcome of pediatric pineoblastoma after surgery, radiation and chemotherapy.
abstract: BACKGROUND
  Pineoblastomas are a category of supratentorial primitive neuroectodermal tumors (sPNETs) occurring in the pineal gland; some studies support the impression that patients with pineoblastomas have a worse prognosis than those with other sPNETs.
  
  
  METHODS
  We reviewed the medical records and tissue sections of all patients with the diagnosis of pineoblastoma that were treated at the Dana-Farber Cancer Institute/Children's Hospital Boston Pediatric Brain Tumor Program between 1986 and 2005.
  
  
  RESULTS
  Thirteen patients with the pathologic diagnosis of pineoblastoma were treated at our Hospital; 11 of these cases had complete records suitable for study. The median age was 8 years 8 months (5 F, 6 M). Surgical, radiation and chemotherapeutic regimens varied from case to case. Three patients had gross total resection and are alive and free of disease, versus four of eight with subtotal resection or biopsy only. Patients who received CSI and multi-agent chemotherapy had improved overall survival.
  
  
  CONCLUSIONS
  Seven of eleven patients with pineoblastoma are currently alive and free of disease, reflecting an improved outcome and longer survival than previously appreciated. Gross total surgical resection appeared to correlate with improved survival, as did treatment with craniospinal irradiation and multi-agent chemotherapy. Further study of this group of patients as a distinct diagnostic entity will be necessary to determine optimal therapy.
authors.affiliation: Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA, USA. gilheens@mskcc.org
authors.forename: Stephen W
authors.initials: SW
authors.lastname: Gilheeney
authors.forename: Ali
authors.initials: A
authors.lastname: Saad
authors.forename: Susan
authors.initials: S
authors.lastname: Chi
authors.forename: Christopher
authors.initials: C
authors.lastname: Turner
authors.forename: Nicole J
authors.initials: NJ
authors.lastname: Ullrich
authors.forename: Liliana
authors.initials: L
authors.lastname: Goumnerova
authors.forename: R Michael
authors.initials: RM
authors.lastname: Scott
authors.forename: Karen
authors.initials: K
authors.lastname: Marcus
authors.forename: Leslie
authors.initials: L
authors.lastname: Lehman
authors.forename: Umberto
authors.initials: U
authors.lastname: De Girolami
authors.forename: Mark W
authors.initials: MW
authors.lastname: Kieran
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D004358:Drug Therapy / Q000706:statistics & numerical data; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D019635:Neurosurgical Procedures / Q000592:standards / Q000706:statistics & numerical data; D010870:Pineal Gland / Q000473:pathology*; D010871:Pinealoma / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D011379:Prognosis; D011878:Radiotherapy / Q000706:statistics & numerical data; D012189:Retrospective Studies; D055502:Secondary Prevention; D015996:Survival Rate; D016896:Treatment Outcome