Gliomatosis cerebri: treatment results with radiotherapy alone

Abstract

Background: Gliomatosis cerebri (GC) is a rare primary brain tumor characterized by proliferation of neoplastic glial cells that typically involve multiple brain areas, with preservation of brain structures and sparing of neurons. The optimal therapeutic strategy is not well established. The impact of radiotherapy on survival in patients with GC remains undefined.

Methods: Between 1980 and 2001, 12 patients with GC were identified, representing less than 1% of all patients with primary brain neoplasms treated at the Cleveland Clinic.

Results: All 12 patients had brain biopsies between March 1986 and July 2001 (seven males, five females, with a median age of 53 years [range, 13-85 years]). Median Karnofsky performance status at the time of presentation was 70 (range, 40-90). Eight patients had low-grade gliomas and four patients had anaplastic astrocytomas. Eight patients received radiation treatment to the brain as the only treatment, and four received neither radiation nor chemotherapy. The median dose of megavoltage radiation was 55.4 Gy (range, 45-61.2 Gy). Of the eight patients who received brain radiotherapy, the clinical and radiologic follow-up findings improved in three patients, stabilized in three patients, and worsened in two patients. Median follow-up was 10.3 months (range, 1-55 months). The median survival for the eight patients who received brain radiotherapy was 11.4 months. The one- and two-year survival rates were 45% and 30%, respectively. Two of the eight patients who received radiotherapy were alive at the time of writing. The four patients who did not receive radiotherapy died of the disease at 0.6, 1.0, 1.9, and 2.4 months after diagnosis.

Conclusions: Gliomatosis cerebri is associated with poor survival. Although brain radiotherapy controlled or stabilized disease progression in most patients, the overall survival after brain radiotherapy alone was not satisfactory. More aggressive therapy may be needed.