ACTR-36. GLIOMATOSIS CEREBRI; CLINICAL COURSE AND TREATMENT OUTCOMES IN SINGLE INSTITUTION

Abstract

Gliomatosis cerebri (GC) reported by Nevin in 1938 is a mysterious and rare primary brain tumor. GC is defined as a diffuse infiltration of neoplastic glial cell at least three lobes in the World Health Organization (WHO) classification and can be categorized with two patterns. Type1 is typical infiltration and has no tumor mass, type2 has tumor mass spreading to CNS involvement. Prognosis of GC is dismal and there is no standard therapeutic regimen for them. Here, we report clinical course in gliomatosis patients at Tokyo Women’s Medical University in Japan. Fourteen GC patients at Tokyo Women’s Medical University were diagnosed by operation or stereotactic biopsy and treated between October 2003 and April 2015. Using retrospective chart review, we analyzed patient demographics, clinical outcome and duration of therapy were estimated using the Kaplan-Meier estimate. GC patients were 10 males and 4 females. The median age was 50 years (range 18–73). Median Overall Survival (mOS),median Progression Free Survival(mPFS) was 30.5 months, 12.5 months and poor OS(grade II, III, IV:38.5,30,8months; P<0.001)and PFS (18.5,17,5months; P<0.001) was related with higher grade. Among Radiation Therapy group(RT),ChemoTherapy group (CT) and Radiation Chemo Therapy group(CRT), CT was related with better prognosis (OS CT, RT, CRT:43, 34, 17months; P<0.05),PFS (24, 12, 8months; P=0.053). Higher histologic grade was associated with poor prognosis. Although radiation therapy was effective in most reported literatures, radiation alone was not effective. Chemotherapy suggested it was possible to be better prognosis.