Gliomatosis cerebri: clinical characteristics, management, and outcomes

Abstract

Gliomatosis cerebri is a rare diffusely infiltrating primary neoplastic glial process of the brain. Our objective is to review clinical presentation, management, and outcome in a large single institution series of gliomatosis cerebri patients. 54 consecutive gliomatosis cerebri cases presenting to Mayo Clinic Rochester between 1991 and 2008 were retrospectively reviewed. Inclusion criteria included involvement of at least three cerebral lobes, lack of a single discrete mass and pathological confirmation of diffuse glioma. Median overall survival (OS) was 18.5 months. Age, gender, presenting symptoms, and contrast enhancement did not correlate significantly with survival, though there was a trend toward decreased overall survival in patients above the median age of 46 years. Karnofsky performance score <70 was associated with poor OS (median 9.5 vs. 20.5 months, p = 0.02). Higher histologic grade was associated with poor progression-free survival (PFS; median for WHO grades II, III, and IV: 21.5, 6.5, and 4 months; p = 0.03) and OS (median 34, 15.5, and 8.5 months; p < 0.05). Radiation therapy was strongly associated with better prognosis (PFS 16.5 vs. 4.5 months, p < 0.01; OS 27.5 vs. 6.5, p < 0.01), but chemotherapy was not. Gliomatosis cerebri patients have a poor prognosis. Lower KPS upon presentation and higher histologic grade predict decreased survival. Surgery's role is limited beyond biopsy for diagnostic purposes. Radiotherapy appears beneficial, although selection bias could be present in this retrospective study. Chemotherapy's value is not as clear but this must be interpreted with caution given variable treatment regimens in this series.

Fig. 1

Representative axial fluid-attenuated inversion recovery (FLAIR) MRI sequences of two patients with gliomatosis cerebri. a Nineteen year old man with a Karnofsky performance score (KPS) of 70 and grade II astrocytoma on biopsy who went on to receive radiation; overall survival 27 months. b Fifty year old man with a KPS of 50 and grade III astrocytoma who did not receive radiation; overall survival 8 months

Fig. 2

Kaplan–Meier survival curves of the 48 patients in our cohort for whom survival data was available. Median overall survival was 18.5 months (a). b Survival curves comparing patients who presented with a high Karnofsky performance score (KPS ≥ 70) and a low KPS (KPS < 70) were significantly different (20.5 vs. 9.5 months, p = 0.02). c Progression free survival curves and d overall survival curves comparing patients with different histologic grades. Median PFS and OS decreased significantly with increasing grade (PFS: 21.5, 6.5, and 4 months for grades II, III, and IV, respectively; p = 0.03. OS: 34, 15.5, and 8.5 months for grades II, III, and IV, respectively; p = 0.04)

Fig. 3

Survival curves with and without treatment. Progression free survival (a) and overall survival (b) were both higher in patients undergoing radiotherapy (PFS: 16.5 vs. 4.5 months, p < 0.01; OS: 27.5 vs. 6.5 months, p < 0.01). Though there was a trend towards improved progression-free (c) and overall survival (d) in the cohort receiving chemotherapy, this difference did not reach statistical significance (PFS: 12.5 vs. 5.5 months, p = 0.67; OS: 21 vs. 11.5 months, p = 0.96)