Radiation is an important component of multimodality therapy for pediatric non-pineal supratentorial primitive neuroectodermal tumors.
OBJECTIVE
To review a historical cohort of pediatric patients with supratentorial primitive neuroectodermal tumors (sPNET), to clarify the role of radiation in the treatment of these tumors.


METHODS
Fifteen children aged <18 years with non-pineal sPNETs diagnosed between 1992 and 2006 were identified. Initial therapy consisted of surgical resection and chemotherapy in all patients and up-front radiotherapy (RT) in 5 patients. Five patients had RT at the time of progression, and 5 received no RT whatever. Kaplan-Meier estimates of overall survival were then calculated.


RESULTS
The median follow-up from diagnosis for all patients was 31 months (range, 0.5-165 months) and for surviving patients was 49 months (range, 10-165). Of the 5 patients who received up-front RT, all were alive without evidence of disease at a median follow-up of 50 months (range, 25-165 months). Only 5 of the 10 patients who did not receive up-front RT were alive at last follow-up. There was a statistically significant difference in overall survival between the patient group that received up-front RT and the group that did not (p = 0.048). In addition, we found a trend toward a statistically significant improvement in overall survival for those patients who received gross total resections (p = 0.10).


CONCLUSIONS
Up-front RT and gross total resection may confer a survival benefit in patients with sPNET. Local failure was the dominant pattern of recurrence. Efforts should be made to determine patients most likely to have local failure exclusively or as a first recurrence, in order to delay or eliminate craniospinal irradiation.
McBride
Sean M
SM
Department of Radiation Oncology, University of California-San Francisco, San Francisco, CA.
Daganzo
Sally M
SM
Banerjee
Anuradha
A
Gupta
Nalin
N
Lamborn
Kathleen R
KR
Prados
Michael D
MD
Berger
Mitchel S
MS
Wara
William M
WM
Haas-Kogan
Daphne A
DA
D017668:Age of Onset; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009367:Neoplasm Staging; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery; D010870:Pineal Gland / Q000473:pathology; D011878:Radiotherapy / Q000379:methods; D013116:Spinal Cord / Q000473:pathology; D033581:Stem Cell Transplantation; D016019:Survival Analysis; D017741:Survivors; D013997:Time Factors