228. Prognostic Factors Associated with Survival Outcomes in Supratentorial Primitive Neuroectodermal Tumors

Authors: Nolan Rea; Richard Gomez, BS; Mark Krieger, MD (Los Angeles, CA)


Supratentorial primitive neuroectodermal tumors (sPNET) are rare but highly malignant neoplasms in pediatric patients. In this study, we investigated prognostic factors associated with patient outcomes.


An IRB-approved, retrospective study was undertaken at a single institution between 1993-2014. Thirty-two patients treated for sPNET’s were identified. Kaplan-Meier survival analyses and log-rank were used to test for statistical significance (p<0.05). Additional factors were analyzed for covariance with cox regression.


Thirty-two patients were identified (22 females, 10 males), with a mean age of 7.3 years. Mean follow up was 6.9 years. Twenty (63%) patients presented with seizures, and the most common tumor locations were frontal (37.5%) and parietal (31.3%). Common radiographic features were calcifications (94%), edema (81%), and diffusion restriction (87%). MRI spectroscopy data confirmed presence of taurine, specifically identified these tumors.  All also had elevated choline levels, indicative of their malignancy. Twenty-five (78%) and 23 (72%) patients received chemotherapy and radiation therapy, respectively. Five mortalities occurred, yielding a 5-year overall survival of 83.9%. All 5 mortalities occurred in subjects who had either recurred or progressed (p=0.01). 5/8 patients with sub-total resection (STR) had tumor progression, whereas 13/24 (54.2%) with GTR had recurrence. Age, adjuvant therapy, seizures, tumor location, and resection type showed no association with survival (p>>0.05). However, patients receiving post-operative radiation therapy were significantly associated with recurrence/progression (p=0.01). Females had notably higher survival compared to males (90.9% vs. 66.7%, p=0.067).


Neither post-resection adjuvant therapy nor resection type significantly affected patient survival, although radiation therapy did show an association with recurrence/progression. Females trended toward increased survival, albeit non-significant. Further analysis with larger sPNET cohorts would better elucidate prognostic factors associated with survival.