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Although clear cells resembling oligodendrocytes are known to occur in ependymomas, tumors composed primarily of such cells, i.e., clear cell ependymoma (CCE), are rare. Herein we characterize the clinicopathologic features of eight examples of CCE encountered at Mayo Clinic from 1983 to 1996. The tumors occurred in patients 3-31 years of age and presented as well-demarcated, deeply situated, contrast-enhancing masses, all of which were supratentorial. All but one case lacked classic light microscopic features of ependymoma. Although nearly all were immunoreactive for glial fibrillary acidic protein, it was electron microscopy that showed the diagnostic hallmarks of ependymoma, including complex intercellular junctions, surface microvilli and cilia, and microrosette formation, thus underscoring the importance of electron microscopy in the diagnosis of clear cell ependymomas. The differential diagnosis of CCE includes not only oligodendroglioma, but central neurocytoma and glioneurocytoma. Unlike oligodendrogliomas, CCEs are characterized by their sharp circumscription, hypervascularity as reflected in contrast enhancement on computed tomography and magnetic resonance imaging, their noninfiltrative pattern of growth that displaces parenchyma, and the occasional formation of vague perivascular pseudorosettes. Unlike central neurocytomas and glioneurocytomas, CCE lack secretory granules, vesicles, and synapses by electron microscopy and neuroendocrine markers by immunocytochemistry. In summary, the diagnosis of CCE requires neuroimaging, histologic, and ultrastructural correlation. The latter is essential in a limited biopsy. Ultrastructural studies also play a role in identifying glioneurocytomas. CCEs behave like more ordinary ependymomas. The importance of their recognition is the avoidance of alternative diagnoses and inappropriate therapies.

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