| Summary: | Clear cell ependymoma (CCE) is an uncommon variant of ependymoma having a predilection for the supratentorial region. Histologically, it bears an uncanny resemblance to oligodendroglioma, central neurocytoma, hemangioblastoma and metastasis from clear cell carcinoma. Here, we report a rare case of clear cell ependymoma in a 45-year-old male, which histomorphologically resembled anaplastic oligodendroglioma on intraoperative smears, frozen section and routine light microscopy. Immunohistochemistry, however, helped to arrive at the correct diagnosis. Unlike other clear cell tumors of the brain parenchyma, CCE is known to follow an aggressive course and, hence, obtaining a correct diagnosis is imperative since it has a direct therapeutic and prognostic connotation.
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