Neglected malignant medulloepithelioma of the eye

Intraocular medulloepithelioma is a rare tumor usually arising from the ciliary body and presenting in childhood. We recently managed a case of an advanced medulloepithelioma in a 45-year-old man that went untreated for several years. The patient had an exenteration after the tumor destroyed the eye and extended into the central nervous system. The initial pathologic diagnosis was complicated by the undifferentiated nature of the tumor which mimicked a lymphoma on fine needle aspiration biopsy (FNAB) as well as on initial permanent sections. The diagnosis was established by immunohistochemical stains indicating a neuroepithelial origin; the relationship of the tumor to the ciliary body and pigmented ciliary epithelium indicated the site of origin.