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Cases Report: We report the cases of 2 women with a pigmented tumour in the ciliary body, one a melanocytoma and the other a melanoma, with different clinical manifestations. The first one presented with decreased visual acuity associated with recent growth of the tumour, as well as sectorial opacities of the lens and subluxation. The second one is asymptomatic and has been kept under observation for more than 30 years., Discussion: Although the definitive diagnosis of a pigmented tumour of the ciliary body is only achieved by the histopathology study, the group of clinical features is a determining factor when a conservative treatment is indicated., (Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.)

Purpose: To discuss a case in which melanocytoma of the ciliary body presented with widespread pigment dispersion and elevated intraocular pressure (IOP)., Methods: A 64-year old woman presented with increased IOP and persistent anterior segment inflammation in her right eye. She had undergone a trabeculectomy for uncontrolled IOP before presentation. Slit-lamp examination, gonioscopy, and ultrasound biomicroscopy revealed a ciliary body mass with extension into the subconjunctiva in addition to widespread pigmentary dispersion in the anterior segment. Enucleation of the right eye was performed for histopathologic evaluation, as a diagnosis of ciliary body melanoma was made on clinical examination., Results: Histopathologic findings were diagnostic of a melanocytoma of the ciliary body with necrosis and focal malignant transformation with extension of melanocytoma cells and melanophages into the subconjunctival space, trabecular meshwork, and anterior chamber angle., Conclusion: Melanocytoma of the ciliary body is a rare intraocular tumor that may present with pigment dispersion and secondary elevated IOP. Careful examination of the anterior segment is imperative in such cases.

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 pigmented mass. Fine-needle aspiration of the iris lesion and a histopathologic study of the subconjunctival 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.

A 59-year-old woman had a sectorial pigmented iris tumor in her right eye and was found to have secondary glaucoma unresponsive to medical therapy. Gonioscopy revealed a satellite tumor in the inferior chamber angle, which we suspected to be a ciliary body melanoma. Subsequently, both lesions were removed completely by superior total and inferior partial iridocyclectomy. Histologic examination disclosed a spindle cell nevus with surface plaque associated with a borderline epithelioid cell nevus. This case is an example of unexpected clinical findings that may be encountered in any iris melanocytic lesion, benign or malignant, and the consequent problems of diagnosis and management.

We present the case of a 22-year-old woman who underwent surgical excision of a malignant melanoma of the skin three years previously and whom we saw for a pigmented tumor of the iridocorneal angle of her right eye associated with end-stage secondary glaucoma. After the clinical diagnosis of ring melanoma, she underwent enucleation of the eye. The pathologic examination showed a lesion predominantly appearing as a nevus with areas of borderline melanoma formation. The main features of this case were the young age of the patient, the predominant nevoid appearance of this diffuse ciliary body tumor with ring configuration, and the fact that it is a second primary melanoma in such a young patient.

Seventeen patients with ocular or oculodermal melanocytosis were identified among 1,250 Caucasian patients with uveal malignant melanoma. The uveal melanomas in these seventeen patients were similar in size, cell type, and tendency to metastasize to those occurring in reported populations without ocular or oculodermal melanocytosis. Clinical and histopathologic study of these 17 cases indicated that all of the affected eyes had episcleral and choroidal melanocytosis and that other ocular and periocular tissues (sclera, iris, conjunctiva, angle structures, and optic disc) were involved less commonly. The uveal malignant melanoma that was present in each of these cases involved the eye with melanocytosis. Furthermore, the melanoma arose from the zone of uveal melanocytosis in every eye with sectoral involvement. Statistical analysis of these data supports the contention that uveal malignant melanoma has a higher incidence in white persons with ocular or oculodermal melanocytosis than in those without these conditions.

The occurrence of uveal and cutaneous malignant melanoma and the dysplastic nevus syndrome in the same individual suggests an etiologic relationship among these diseases. Thus, the dysplastic nevus syndrome could be viewed as marking an increased risk of both cutaneous and ocular melanoma. We postulated that if such a relationship exists, patients with both forms of melanoma should have a high prevalence of dysplastic nevi. We examined 44 patients (31 women and 13 men ranging in age from 20 to 80 years) with uveal melanoma for evidence of cutaneous melanoma and dysplastic nevi. We also examined photographs of 46 patients (24 men and 22 women ranging in age from 19 to 67 years) with nonfamilial cutaneous melanoma to determine the prevalence of dysplastic nevi. We found a 4.5% prevalence of dysplastic nevi in patients with uveal melanoma, significantly lower than the 41% prevalence in patients with cutaneous melanoma (two of 44 patients vs 19 of 46 patients). This study indicates that uveal and cutaneous melanoma are not etiologically linked through dysplastic nevi and suggests that patients with uveal melanoma are no more likely to have cutaneous dysplastic nevi than the general population.