MS-10 * CLEAR CELL EPENDYMOMA: A RARE VARIANT FIRST TIME REPORTED BY A TERTIARY CARE HOSPITAL IN PAKISTAN

Abstract

OBJECTIVES: Clear Cell Ependymoma has never been reported from Pakistan. We aim to see the features and outcomes of this rare entity in Pakistan. METHODS: A retrospective case-series conducted at the Department of Neurosurgery, Aga Khan University. The medical record files of patients with Clear Cell Ependymoma from 2003 - 2012 were reviewed, MRI/C.T scans and Histopathological slides were reviewed. Patients lost to follow-up were contacted on telephone. Analysis was done on SPSS 20. RESULTS: Nine cases of CCE were found. The median age was 47 years. The symptoms included Headache 66.6%, seizures, hemiparesis, raised ICP 33.3% patients. Seven patients had supra-tentorial and 2 had infra-tentorial tumor. MRI showed hypo-intense signals on T1 and hyper-intense signals on T2 weighted images. Contrast enhancement was found in 7 patients (77.8%). Histopathology: Clear cells were found frequently in 5 cases. Immunohistochemistry showed GFAP and EMA positivity in all cases. Ki-67 showed high proliferative index in 5 patients. WHO class was II in 1 patient and III in 8 patients. Gross total resection was done in 5 (55.5%), Subtotal Resection in 4 patients (44.4%). 5 patients underwent radiotherapy, 3 patients underwent Radiotherapy + chemotherapy, post-surgery. Recurrence was there in 7 patients. Repeat surgery was done in 3 patients. Six patients died of the disease. Median Progression free survival was 8 months (range 5-28 months), the median overall survival was 15 months (range 6-52months). CONCLUSION: CCE is a significantly aggressive tumor in Pakistan with prognosis worse than other parts of the world.