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.
Similar content being viewed by others
References
Bietti G (1931) Ueber ein vom Pigmentepithel ausgehendes Melanokarzinom des Bulbus und der Orbita. Klin Monatsbl Augenheilk 87:459–486
Byrne SF (1986) Standardized echography of the eye and orbit. Neuroradiology 28:618–626
Duke JR, Maumenee E (1959) An unusual tumor of the retinal pigment epithelium in an eye with early open-angle glaucoma. Am J Ophthalmol 47:311–317
Earle J, Kline RW, Robertson DM (1987) Selection of iodine-125 for the Collaborative Ocular Melanoma Study. Arch Ophthalmol 105:763–764
Fair JR (1958) Tumors of the RPE. Am J Ophthalmol 45:495–504
Fan JT, Robertson DM, Campbell RJ (1995) Clinicopathologic correlation of a case of adenocarcinoma of the retinal pigment epithelium. Am J Ophthalmology 119:243–245
Finger PT (1992) Microwave plaque thermoradiotherapy for choroidal melanoma. Br J Ophthalmol 76:358–364
Finger PT, Moshfeghi DM, Ho TK (1991) Palladium-103 ophthalmic plaque radiotherapy. Arch Ophthalmol 109:1610–1613
Finger PT, Lu D, Buffa A, Bosworth JL (1993) Palladium-103 Versus Iodine-125 For Ophthalmic Plaque Radiotherapy. Int J Radiat Oncol Biol Phys 27:849–854
Finger PT, Buffa A, Mishra S, Berson A, Bosworth JL, Vikram B (1994) Palladium-103 plaque radiotherapy for uveal melanoma: Clinical experience. Ophthalmology 101:256–263
Font RL, Zimmerman LE, Fine BS (1972) Adenoma of the retinal pigment epithelium: histochemical and electron microscopic observations. Am J Ophthalmol 73:544–554
Garner A (1970) Tumours of the retinal pigment epithelium. Br J Ophthalmol 54:715–723
Greer CH (1952) Epithelial tumors of the retinal pigment epithelium. Trans Ophthalmol Soc UK 72:265–277
Griffith J (1894) On a rare form of primary intraocular melanoma. Trans Ophthalmol Soc UK 14:160–166
Kurz GH, Zimmerman LE (1962) Vagaries of the retinal pigment epithelium. Int Ophthalmol Clin 2:441–464
Martens M (1921) Eine primare bosartige epitheliale Geschwulst des Augeninnern beim Erwachsenen. Arch Augenheilkd 89:1–22
Minckler D, Allen AW (1978) Adenocarcinoma of the retinal pigment epithelium. Arch Ophthalmol 96:2252–2254
Reese AB (1953) Epithelial tumors. In: Tumors of the eye. Harper, New York, pp 62–63
Reese AB, Jones IS (1956) Benign melanomas of the retinal pigment epithelium. Am J Ophtalmol 42:207–212
Ronne G (1945) Malignant tumour originated from the pigment epithelium of the retina. Acta Ophthalmol 23:382–383
Schuster H (1928) Retinoepithelioma malignum pigmentosum. Virchows Arch Pathol Anat Physiol Klin Med 269:97–104
Theobald GD, Floyd G, Kirk HQ (1958) Hyperplasia of the retinal pigment epithelium: simulating a neoplasm: report of two cases. Am J Ophthalmol 45:235–240
Tso MOM, Albert DM (1972) Pathologic conditions of the retinal pigment epithelium. Arch Ophthalmol 88:27–38
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Finger, P.T., McCormick, S.A., Davidian, M. et al. Adenocarcinoma of the retinal pigment epithelium: A diagnostic and therapeutic challenge. Graefe's Arch Clin Exp Ophthalmol 234 (Suppl 1), S22–S27 (1996). https://doi.org/10.1007/BF02343044
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02343044