Clinical diagnosis of gliomatosis cerebri: Report of three cases

Abstract

Distinction of gliomatosis cerebri (GC), a rare entity characterized by a widespread infiltration of the brain by tumor, from diffuse glioma is a difficult clinical problem. Most previously reported cases of GC have been autopsy cases because of the lack of objective and quantitative clinical diagnostic criteria. In order to better define this entity, we report the neuroradiological and pathological findings of three cases of GC. Three patients (one man and two women, aged 46-71 years) presented with symptoms of mild increased intracranial pressure, cognitive impairment, or seizure. Magnetic resonance imaging (MRI) was done with T1-weighted images after gadolinium injection, and with T2-weighted images and fluid attenuated inversion recovery (FLAIR) in all cases. Histological confirmation of glial proliferation was obtained in all cases by craniotomy. The topography of the tumoral infiltration was characteristic, involving mainly the white matter, basal ganglia, thalamus, and commissural fibers. More than two cerebral lobes were affected. Contrast enhancement was absent, and mass effects were minimal compared with the extent of tumoral infiltration, but one patient presented with a small frontal enhanced mass during the clinical course. The pathological analyses demonstrated infiltration of the brains by variably differentiated neoplastic glial cells with destruction of the myelin sheath, but the involved axis cylinder and neuronal cells were preserved. Diagnosis of GC should be faithful to the pathological diagnosis criteria of Scheinker and Evans, and therefore the precise assessment of MRI findings according to these criteria is required for clinical, antemortem diagnosis of GC.