Gliomatosis Cerebri: Clinical Features, Treatment, and Prognosis

Summary  To clarify clinical features and to elucidate prognostic factors and prognosis, the authors retrospectively analyzed 16 cases of gliomatosis cerebri treated at Seoul National University Hospital between January 1988 and December 1995. Age at diagnosis ranged from 19 to 62 (median 34) years and male to female ratio was 10:6. Most presented with headache or seizure, and the mean duration of symptoms was 12.8 months. A poorly defined diffuse high signal intensity lesion, extending in T2-weighted images for two lobes or more, was the characteristic magnetic resonance (MR) image finding. On postcontrast T1-weighted MR imaging, focal enhancement of the lesion was detected in five cases. All patients underwent histological confirmation by craniotomy (9 cases) or stereotactic biopsy (7 cases). Histologically, all patients had compatible findings of gliomatosis cerebri which are the widespread infiltration of neoplastic glial cells with minimal destruction of pre-existing structures.  After histological diagnosis, external radiation therapy was begun except in one case, who declined this treatment. Fourteen patients completed the whole procedure and received the planned dose (mean 5780 cGy). Median survival time after diagnosis was 38.4 months. In univariate analysis, the Ki-67 labelling index (>1) showed significant correlation with the length of survival (p=0.006).  It is suggested that 1) gliomatosis cerebri can be diagnosed by a combination of MR imaging findings and histological examination; 2) histological diagnosis and external radiation therapy might be a good treatment modality; 3) the Ki-67 labelling index correlates significantly with survival time.