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 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.


CONCLUSIONS
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.
Finger
P T
PT
Department of Ophthalmology, New York Eye and Ear Infirmary, New York, USA. PaulFinger@aol.com
McCormick
S A
SA
Davidian
M
M
Walsh
J B
JB
D000230:Adenocarcinoma / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000532:radiotherapy; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001918:Brachytherapy; D003937:Diagnosis, Differential; D015353:Eye Enucleation; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009887:Ophthalmoscopy; D010165:Palladium / Q000627:therapeutic use; D010857:Pigment Epithelium of Eye / Q000000981:diagnostic imaging / Q000473:pathology* / Q000528:radiation effects; D011868:Radioisotopes / Q000627:therapeutic use; D012164:Retinal Diseases / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000532:radiotherapy; D014463:Ultrasonography
D011868:Radioisotopes; D010165:Palladium