Gliomatosis cerebri: a review of 22 cases

Abstract

Objective: Gliomatosis cerebri is an enigmatic diffuse brain neoplasm whose prognosis is grim. We reviewed data for patients with gliomatosis who were treated at the University of California, San Francisco, during a 10-year period. Our focus was on presentation, radiological and pathological features, and outcomes.

Methods: We reviewed hospital and clinic records and magnetic resonance imaging scans for 22 patients with gliomatosis. The diagnosis was based on magnetic resonance imaging findings and tissue confirmation for all patients. Seven patients also underwent magnetic resonance spectroscopy. Eleven patients were male (50%), and the median age at presentation was 49 years (range, 7-79 yr).

Results: Kaplan-Meier analysis demonstrated median lengths of survival as follows: no treatment, 1 month (n = 4); radiotherapy alone, 28 months (95% confidence interval, 5-51 mo; n = 13); radiotherapy followed by chemotherapy, two patients, alive at 28 and 104 months; radiotherapy and chemotherapy simultaneously, three patients, one alive at 18 months and the others dead at 7 and 9 months. There was no significant difference between radiotherapy alone and radiotherapy combined with chemotherapy (P = 0.69). Karnofsky Performance Scale scores of >/=70 and grade were both significantly related to length of survival in univariate analyses (P < 0.05); these correlations were confirmed in the multivariate analysis, although the small numbers of patients and deaths precluded reliable interpretation.

Conclusion: Although the small number of patients in our study and its retrospective nature preclude definitive conclusions regarding the utility of treatment, our findings suggest that biopsies are useful not only for diagnosis but also for prediction of the length of survival.