ADENOCARCINOMA OF THE RETINAL-PIGMENT EPITHELIUM - A DIAGNOSTIC AND THERAPEUTIC CHALLENGE

Background: Adenocarcinoma of the retinal pigment epithelium (RPE) is an uncommon intraocular tumor which has been rarely if ever diagnosed prior to enucleation. Our review of the literature suggests that when a melanotic and tuberous-shaped tumor presents in a woman with signs o f uveitis one should consider the diagnosis of adenocarcinoma of the R PE. Methods: We report a case in which a 4.9-mm-tall, mushroom-shaped, moderately reflective melanotic tumor found to be present in a 50-yea r-old woman. Other findings included a 12X10-mm dense transilluminatio n shadow and 270 deg of posterior iris synechiae. With a presumptive d iagnosis of uveal melanoma the patient was treated with palladium-103 plaque radiotherapy. Within 6 months she underwent enucleation due to uncontrollable ocular pain and progressive tumor growth. Histopatholog ic evaluation revealed an adenocarcinoma of the (RPE). Results: Two mo nths after radiotherapy the eye developed acute angle-closure glaucoma , secondary retinal detachment, and a painful scleritis (a result simi lar to that of Greer, who treated an intraocular adenoma with radiatio n). Our diagnosis of adenocarcinoma of the RPE was made after enucleat ion. Conclusion: Adenocarcinomas of the RPE are rarely if ever diagnos ed on clinical evaluation, should be suspected in women with a melanot ic intraocular tumor and uveitis, and may respond poorly to ophthalmic plaque radiotherapy. All reported cases presenting without extrascler al extension have not metastasized.