Gliomatosis CerebriClinical Features and Prognostic Factors of Long-term Survival

ABSTRACT

INTRODUCTION: The authors present a retrospective analysis of the clinical features and prognostic factors of long-term survival in gliomatosis cerebri. METHODS: The authors reviewed the clinical features of 27 cases of gliomatosis cerebri treated between August 1988 and January 2001. Age at diagnosis ranged from 19 to 62(median 41) years and the male to female ratio was 18:9. Most cases presented as a headache or seizure and the mean duration of symptoms was 9.6 months. An ill defined, diffuse high signal intensity lesion extending two lobes or more, without a central necrotic center in T2-weighted magnetic resonance(MR) imaging was characteristic. All patients underwent histological confirmation by craniotomy(12 cases), stereotactic biopsy(14 cases) and stereotactic biopsy followed by craniotomy(1 case). External beam radiation therapy was administered in every case except two, one of which expired within a month of surgery and the other refused treatment. RESULTS: Mean survival time after diagnosis was 41.1 months. By univariate analysis, the symptom duration(lower than 12 months) and the Karnofsky performance scale at discharge(lower than 70), focal enhancement on preoperative MR imaging, postoperative increased intracranial pressure sign, pathologic grade(high grade) and p53(>5%) were correlated with the length of survival(p-values were 0.07, 0.00, 0.007, 0.001, 0.04, and 0.02 respectively). CONCLUSION: We suggest that:1) gliomatosis cerebri can be diagnosed by a combination of MR imaging and histopathological examination:2) confusion with extensive glioma is possible due to vague diagnostic criteria, and so survival might be better than expected had discrete diagnosis been made. 3) Initial active management for increased ICP and further radiation therapy might be an important therapy.