FOCAL MIDBRAIN GLIOMA - LONG-TERM SURVIVAL IN A COHORT OF 16 PATIENTSAND THE IMPLICATIONS FOR MANAGEMENT

Background: Focal gliomas involving the midbrain tectum and tegmentum have been identified as having a better prognosis than diffuse tumors affecting the brain stem. However, only limited information is availab le concerning treatment effectiveness and long term outcome for these patients. Methods: A retrospective, population-based cancer registry s urvey was performed to assess the clinical features and treatment cour ses of patients with focal midbrain tumors. Results: Sixteen patients with midbrain gliomas were identified; eight had tectal gliomas and ei ght tegmental gliomas. Thirteen patients presented with symptoms relat ed to hydrocephalus, and 12 required a ventriculoperitoneal shunt. Sev en patients underwent surgery directed at the tumor. Eight patients un derwent initial radiation therapy and none had initial chemotherapy. O ne patient diagnosed at age 18 months had a rapidly growing tumor afte r 14 months of follow up which as responded to chemotherapy. The mean survival of this patient population was 84 months (range 3-280 months) after diagnosis, with only one tumor related death occurring (280 mon ths after diagnosis). Survival was not affected by tumor location with in the midbrain (tegmental or tectal) or by whether radiation therapy was or was not administered. Conclusions: Patients with focal midbrain gliomas require symptom control aimed at treatment of hydrocephalus, or mass effect from the tumor. However the extended survival of this p opulation suggests that routine aggressive surgical debulking is often not required. Furthermore, the routine use radiation therapy or chemo therapy for all such patients is questioned.