Prognostic implications of histological clear cells in high-grade intracranial ependymal tumors: A retrospective analysis from a tertiary care hospital in Pakistan

Background: Clear cell variant in ependymal tumors is rare. We aimed to compare the features and outcome of the World Health Organization (WHO) Grade 3 ependymal tumors with clear cells to the WHO Grade 3 classic anaplastic ependymoma (AE). Materials and Methods: A retrospective cohort study conducted at the Department of Neurosurgery, Aga Khan University, Pakistan, from 2003 to 2013. The medical records and radiology of patients with proven histopathology were reviewed. The analysis was done on SPSS 20. Results: Nine cases of clear cell variant and 23 cases of classic AE were found, both of which combined equated to 4% of the total tumor burden in 11 years. The median age of clear cell ependymoma (CCE) and AE were 49 and 37 years, respectively. Presenting symptoms included headache in 66% of CCE and 63% in AE, raised intracranial pressure accounting for 33% of CCE and 54% of AE, dizziness in 22% CCE and 39% AE, while seizures presented equally in both. Supratentorial location was observed in 77% CCE and 48% AE. Both showed hypointense signals on T1-weighted images of magnetic resonance imaging (MRI) while T2-weighted images showed hyperintensity in all cases of CCE but only 80% of AE. MRI characteristics such as the presence of cystic component were found in 89% of CCE and 68% of AE, necrosis in 33% CCE, and 22% AE, hemorrhagic in 22% CCE and 9% AE, and equivalent contrast enhancement. Gross total resection was achieved in 5 (55%) patients of CCE, compared to 6 (26%) patients in AE. Subtotal resection was done in 4 (44%) patients of CCE and 15 (65%) patients of AE. Radiotherapy was given to 5 (55%) patients of CCE and 12 (52%) patients of AE. Recurrence was observed in 77% cases of CCE and 70% of AE, with metastasis in 29% of AE and in only one patient of CCE. Repeat surgery was done in 3 (33%) cases of CCE and 8 (35%) cases of AE. Median progression-free survival and overall survival were 9 and 13 months, respectively, in CCE while 14 and 18 months, respectively, for AE. Conclusion: Clear cells in the WHO Grade 3 ependymal tumors are found mainly in a comparatively older adult population with a predilection for supratentorial location and are more aggressive in behavior with poorer outcome than AE.

Key-words:

Anaplastic ependymoma - clear cell ependymoma - progression-free survival - recurrence - rosettes