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

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.

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.

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.

Patients with primary ocular melanoma have an increased risk to develop cutaneous melanoma and should therefore be examined regularly by dermatologists.