Date of Award
Open Access Thesis
Medical Doctor (MD)
Purpose: We reviewed a historical cohort of pediatric patients with supratentorial primitive neuroectodermal tumors (sPNET) in order to clarify the role of radiation in the treatment of these tumors. Patients and Methods: Fifteen children <18 years old with non-pineal sPNETs diagnosed between 1992 and 2006 were identified. Initial therapy consisted of surgical resection and chemotherapy (CT) in all patients and up-front radiotherapy (RT) in 5 patients. Five patients had RT at the time of progression and five received no RT whatsoever. Kaplan-Meier estimates of overall-survival (OS) were then calculated. Results: The median follow-up from diagnosis for all patients was 31 months (range 0.5-165) 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). 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 group of patients that received up-front RT and the group that did not (P=0.048). Additionally, we found a trend toward a statistically significant improvement in overall-survival for those patients that received gross total resections (P=0.10). Conclusions: Up-front radiotherapy 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 cranio-spinal irradiation (CSI).
McBride, Sean, "Radiation is an Important Component of Multimodality Therapy for Pediatric Supratentorial Non-Pineal Neuroectodermal Tumors" (2009). Yale Medicine Thesis Digital Library. 441.