Prognostic factors for patients with gliomatosis cerebri: retrospective analysis of 17 consecutive cases.

Gliomatosis cerebri (GC) is a rare fatal glial neoplasm of the central nervous system. Neuroimaging, histological, and clinical outcome data were reviewed of 17 consecutive patients, 8 males and 9 females aged 15-68 years (median, 37 years), treated for GC between April 1992 and October 2007. All patients received cranial irradiation to include the hyperintense area on T2-weighted magnetic resonance imaging. The total dose of the radiotherapy was 50-72 Gy (median, 60). Intravenous nimustine hydrochloride was administered in all patients, combined with temozolomide in four patients. The median survival time was 23.3 months, with a median follow-up of 23.3 months. Kaplan-Meier analysis demonstrated the overall survival rate which was 70.6% for 1 year, 23.5% for 3 years, and 17.7% for 5 years. Spinal enhanced lesions and nodular malignant transformation to glioblastoma were observed during follow-up in two patients each. Poor survival showed correlation with higher Ki-67 labeling index, higher choline/N-acetylaspartate ratio on magnetic resonance spectroscopy, tumor volumes, lower Karnofsky performance status on admission, cognitive/behavioral deterioration, poor response to the initial radiochemotherapy, and emergence of paraventricular enhanced lesions during the clinical course. The prognosis for patients with GC is unfavorable, but radiochemotherapy may prolong survival.