Ocular melanoma: Epidemiology, clinical presentation and relationship with dysplastic nevi

Purpose: Ocular melanoma is a rare entity compared to cutaneous malignant melanoma. We examined the frequency of the tumor in a defined geographic region, its clinical presentation and its relationship with dysplastic nevi in 136 patients. Methods: 136 patients ( 64 men and 72 women; mean age 61.7 years, range 20 - 92 years) with ocular melanoma were treated at the University Hospital of Graz between June 1996 and December 2001. 129 had primary uveal melanoma in one eye ( 117 choroidal melanomas, 11 melanomas of the ciliary body and 1 of the iris), 2 patients had uveal melanoma in both eyes, 4 patients had conjunctival melanoma and 1 patient had a melanoma of the lacrimal sac. Epidemiology, history, potential risk factors, clinical presentation and relationship with dysplastic (= atypical) nevi were documented. Results: 48 patients ( 35.3%) showed more than five dysplastic nevi, compared to only 1.2% in the general population (chi(2) test: p < 0.001). 5 (3.7%) had additional cutaneous melanoma and 7 ( 5.1%) had a family history of melanoma. The lifelong risk for the occurrence of an additional primary cutaneous melanoma was 2.9%, which is significantly higher than the usual estimate of 1% for the general population. Conclusions: Patients with primary ocular melanoma have an increased risk to develop cutaneous melanoma and should therefore be examined regularly by dermatologists. Copyright (C) 2004 S. Karger AG, Basel.