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A Recently Recognized Syndrome

Charles C. Barr, MD; Lorenz E. Zimmerman, MD; Victor T. Curtin, MD; Ramon L. Font, MD

Arch Ophthalmol. 1982;100(2):249-255.

Abstract
• Four cases of the simultaneous occurrence of bilateral diffuse melanocytic uveal tumors were studied by us. In each case, the patient had an associated systemic malignant neoplasm diagnosed by biopsy or at autopsy. There was no evidence of metastatic melanoma in any patient. Clinically, the patients experienced rapid loss of vision, and cataracts were usually present. Multiple pigmented and nonpigmented placoid iridic and choroidal nodules, as well as serous retinal detachment, frequently were noted. Histopathologically, the uveal tracts of both eyes were diffusely infiltrated by preponderantly benign-appearing nevoid or spindle-shaped cells. Areas of necrosis within the tumors and scleral involvement were commonly seen. We believe this constellation of findings constitutes a new syndrome in which there is a bilateral, diffuse proliferation of melanocytic cells throughout the uvea in association with a systemic malignant neoplasm.

Author Affiliations
From the Registry of Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC (Drs Barr, Zimmerman, and Font); and the Francis Bascom Palmer Eye Institute, Miami (Drs Barr and Curtin).

Footnotes
Accepted for publication March 21, 1981.

The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense.

Reprint requests to the Department of Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306 (Dr Zimmerman).

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