NI-63GLIOMATOSIS CEREBRI: DIAGNOSIS, MANAGEMENT AND OUTCOMES IN THE LARGEST SERIES FROM A SINGLE EUROPEAN INSTITUTION

BACKGROUND AND PURPOSE: Gliomatosis cerebri (GC) is a rare diffusely infiltrating primary glial neoplasm, which is defined as involving at least three lobes of the brain. There often is a striking discordance between clinical presentation and radiological findings. We reviewed the clinical presentation, radiographic appearance and outcome measures from a large single institution series of pathologically defined gliomatosis cerebri cases. METHODS: Between 1992 and 2014, 46 histologically proven cases with GC were encountered. Demographics, initial clinical presentation incl. Karnofsky index (KPS), imaging findings and treatment regimen were retrospectively reviewed and analyzed. RESULTS: Of 46 patients, only eleven were female (23.9%). Mean age at diagnosis was 50.7 years. Most frequent clinical signs were headaches, seizures and mental status changes. CT imaging revealed hypoattenuation of the involved brain parenchyma in 42 of the 46 cases (91.3%) and isodense parenchyma in 4 cases. Magnetic resonance imaging (MRI) generally showed T1-weighted hypointensity and T2-weighted hyperintensity. Frequently bilateral invasion with involvement of the anterior white commissure, the corpus callosum or both was observed. The extent of brain involvement did not demonstrate a significant correlation with overall survival (OS). Lower KPS upon presentation and higher histologic grade did predict shorter OS.