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

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

Introduction:

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.

Methods:

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.

Results:

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).

Conclusion:

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.