Outcome of pediatric pineoblastoma after surgery, radiation and chemotherapy.
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.
Gilheeney
Stephen W
SW
Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA, USA. gilheens@mskcc.org
Saad
Ali
A
Chi
Susan
S
Turner
Christopher
C
Ullrich
Nicole J
NJ
Goumnerova
Liliana
L
Scott
R Michael
RM
Marcus
Karen
K
Lehman
Leslie
L
De Girolami
Umberto
U
Kieran
Mark W
MW
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