Gliomatosis cerebri: review of 22 patients

Introduction: Gliomatosis cerebri is a diffuse astrocytic neoplasm that involves more than two lobes of the brain. Treatment is not well defined and the prognosis is considered poor. Methods: Retrospective analysis of 22 patients with gliomatosis cerebri. Results: We identified 17 men and 5 women (median age 54 years) seen in a Division of Neuro-oncology over a 6 year period. Patients presented with focal sensorimotor or visual deficits (86.4%), seizures (36.4%), cognitive dysfunction (27.3%), or headache (27.3%), suggesting in some cases stroke, migraine, or limbic encephalitis. All patients had bilateral involvement; the regions involved included, temporal (19), basal ganglia (18), frontal (17), parietal (17), corpus callosum (10), and occipital (9). The most frequent pathological findings were grade III astrocytoma (36.4%), grade II astrocytoma (22.7%), and grade IV astrocytoma (18.3%). Nine patients were diagnosed within the first month of symptom development, 11 between the first month and 1 year, and 2 after one year. Seventeen patients received treatment with chemotherapy, radiotherapy or both, and 12 patients (70.6%) had a clinical or radiological response. The median follow-up was 13 months, median progression free survival 6 months, and median survival 9,5 months (15 months if the patients received treatment). Eight patients had thromboembolic events. Conclusions: Gliomatosis cerebri has a variable clinical course. Treatment often results in clinical responses. In this study de median survival of patients who received treatment was similar to that reported in series of glioblastoma multiforme. (C) 2009 Sociedad Espanola de Neurologia. Published by Elsevier Espana, S.L. All rights reserved.