Adenocarcinoma of the Retinal Pigment Epithelium: A diagnostic and therapeutic challenge

Paul T. Finger, Steven A. McCormick, Mary Davidian, Joseph B. Walsh

Research output: Contribution to journalArticlepeer-review

29 Scopus citations

Abstract

• 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 of uveitis one should consider the diagnosis of adenocarcinoma of the RPE. • 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-year-old woman. Other findings included a 12X10-mm dense transillumination shadow and 270 deg of posterior iris synechiae. With a presumptive diagnosis 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. Histopathologic evaluation revealed an adenocarcinoma of the (RPE). • Results: Two months after radiotherapy the eye developed acute angle-closure glaucoma, secondary retinal detachment, and a painful scleritis (a result similar to that of Greer, who treated an intraocular adenoma with radiation). Our diagnosis of adenocarcinoma of the RPE was made after enucleation. • Conclusion: Adenocarcinomas of the RPE are rarely if ever diagnosed on clinical evaluation, should be suspected in women with a melanotic intraocular tumor and uveitis, and may respond poorly to ophthalmic plaque radiotherapy. All reported cases presenting without extrascleral extension have not metastasized.

Original languageEnglish
Pages (from-to)S22-S27
JournalGraefe's Archive for Clinical and Experimental Ophthalmology
Volume234
Issue numberSUPPL. 1
DOIs
StatePublished - 1996
Externally publishedYes

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