PRESUMED ADENOCARCINOMA OF THE RETINAL-PIGMENT EPITHELIUM IN A BLIND EYE WITH A STAPHYLOMA

The retinal pigment epithelium (RPE) can undergo reactive hyperplasia and metaplasia following a variety of ocular insults. However, true ne oplasms of the RPE are rare. We report a case of a papillary adenocarc inoma of the RPE arising in the blind staphylomatous right eye of a 79 -year-old woman with a long history of bilateral posterior staphylomas who was seen with increasing pain and exophthalmos of the right eye. Findings from ultrasonography and computed tomography demonstrated lin ear calcification consistent with osseous metaplasia of the RPE. Progr ession of the exophthalmos and worsening exposure keratitis led to enu cleation of the eye. Gross pathology showed a 79-mm-long globe. Histop athologic findings revealed a largely amelanotic papillary adenocarcin oma arising from the RPE. Positive immunoreactivity for cytokeratin su pported the epithelial origin of the tumor. Adenocarcinoma of the RPE is rare but may develop in a blind eye.