Adenocarcinoma of the retinal pigment epithelium: a diagnostic and therapeutic challenge.

Journal Article (Journal Article;Review)

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 12 x 10-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.

Full Text

Duke Authors

Cited Authors

  • Finger, PT; McCormick, SA; Davidian, M; Walsh, JB

Published Date

  • August 1996

Published In

Volume / Issue

  • 234 Suppl 1 /

Start / End Page

  • S22 - S27

PubMed ID

  • 8871146

International Standard Serial Number (ISSN)

  • 0721-832X

Digital Object Identifier (DOI)

  • 10.1007/BF02343044


  • eng

Conference Location

  • Germany