378 CLINICAL PATHOLOGIC REVIEW DAVID APPLE AND MILTON BONIUK, EDITORS SURVEY OF OPHTHALMOLOGY VOLUME 42 • NUMBER 4 • JANUARY–FEBRUARY 1998 © 1998 by Elsevier Science Inc. 0039-6257/98/$19.00 All rights reserved. PII S0039-6257(97)00087-8 Diffuse Melanotic Lesion of the Iris as a Presenting Feature of Ciliary Body Melanocytoma: Report of a Case and Review of the Literature JYOTIRMAY BISWAS, MS, CANDACE D’SOUZA, MBBS, AND MAHESH P. SHANMUGAM, DO Medical and Vision Research Foundations, Madras, India Abstract. Melanocytoma of the ciliary body is quite rare. Although the tumor is benign, it can spread to the surrounding structures, causing glaucoma and giving an erroneous impression of melanoma. We report a case of melanocytoma of the ciliary body in a 19-year-old woman who presented with diffuse melanocytic proliferation of the iris with uncontrolled secondary glaucoma and a subconjunctival pig- mented mass. Fine-needle aspiration of the iris lesion and a histopathologic study of the subconjuncti- val mass could not rule out melanoma. Enucleation of the painful glaucomatous eye showed evidence of ciliary body melanocytoma with extension into the angle structures as well as the subconjunctival tissue. ( Surv Ophthalmol 42 :378–382, 1998. © 1998 by Elsevier Science Inc. All rights reserved.) Key words. ciliary body • cytology • fine-needle aspiration biopsy • melanocytoma • melanoma • secondary glaucoma A clinical diagnosis of melanocytoma of the ciliary body is often difficult, as this entity is rare, and diag- nosis becomes more difficult when the melanocy- toma extends to the surrounding structures. It can also mimic a melanotic tumor of the iris. Growth of a pigmented lesion and the development of secondary glaucoma indicate that the lesion is a malignant tu- mor or a malignant transformation of a benign mel- anocytic lesion. In the literature there are only a few reports of cil- iary body melanocytoma. 1,2,4,5,7,9–14,16 Frangieh and coworkers 2 provided a review of 23 cases in 1985. In the majority of cases, a diagnosis of melanocytoma was made only on pathologic examination of the eyeball which was removed due to suspicion of mela- noma. The mean age of the patients was 50 years, ranging from 3 months to 90 years. Melanocytoma of the uveal tract can mimic melanomas, particularly when such tumors undergo necrosis, causing glau- coma, or when they spread to the surrounding tis- sue, e.g., iris root, trabecular meshwork, episclera, and sclera. Necrosis or a malignant transformation of this benign tumor causes a diagnostic dilemma. Various ancillary tests, such as ultrasound, phos- phorus-32 uptake, and fluorescein angiography, do not help the clinician to rule out such malignant changes. We report a patient with histopathologi- cally proven melanocytoma of the ciliary body who presented with diffuse melanocytic proliferation of the iris, with uncontrolled secondary glaucoma and a subconjunctival pigmentary mass that mimicked an iris malignant melanoma. Fine-needle aspiration biopsy of the lesion could not rule out malignancy and the patient ultimately underwent enucleation.