Medulloepithelioma of the ciliary body and optic nerve: Clinicopathologic, CT, and MR imaging features

The definitive diagnosis of medulloepitheliomas of the ciliary body and optic nerve is made by histopathologic examination. Familiarity with rare tumors aids clinicians in accurately diagnosing this rare neoplasm. CT and MR imaging findings can be helpful, especially if the mass is confined in an area of the ciliary body without involvement of the retina. The characteristic findings of cystic changes and possible calcification are suggestive of medulloepithelioma rather than melanoma. The more common and ominous retinoblastoma shares some radiologic features with medulloepithelioma, however, and is thus often a consideration. Clinical diagnosis of medulloepitheliomas of the optic nerve and CNS is more challenging. The diagnosis is often made by histopathologic examination. Lastly, coexisting CNS tumors and CNS anomalies have been reported in association with intraocular medulloepitheliomas and should be recognized.