Gliomatosis cerebri: findings with computed tomography and magnetic resonance imaging

Gliomatosis cerebri (GC), is a rare neoplastic disease (less than 150 cases reported in the literature) with a diffuse, widespread proliferation of neoplastic glial cells in the brain, generally affecting both hemispheres and involving the gray and white matter. Less commonly, the cerebellum, the brain stem and the medulla can be affected. Histologic evaluation reveals neoplastic astrocytes with varying levels of differentiation. Perineuronal and perivascular spread of tumor infiltration is observed. Demyelination can be found in the affected areas. A well-preserved underlying neuroanatomic architecture is considered characteristic. Clinical signs vary and are non-specific, including changes in the mental state and headaches, followed by focal motor deficits and convulsive episodes. The prognosis is poor, ranging from weeks to some years after the manifestation of the symptoms. Steroids may be useful in the short term, but chemotherapy is of little value and radiotherapy of questionable benefit. The literature was reviewed and the radiological pattern of three new cases of GC is reported. In two cases the diagnosis was achieved ante-mortem. (Copyright (c) 1998 Elsevier Science B.V., Amsterdam. All rights reserved.)