High Risk of Malignant Melanoma in Melanoma-Prone Families with Dysplastic Nevi

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Abstract
The risk of hereditary cutaneous malignant melanoma was evaluated in 401 members of 14 families with an autosomal dominant form of melanoma. Primary melanoma (127) in 69 family members were documented, including 39 new melanomas diagnosed in 22 study participants from the time of 1st examination through a maximum of 8 yr of follow-up. The 39 newly diagnosed melanomas occurred only in family members with dysplastic nevi, a known precursor of familial melanoma. Of 77 patients with dysplastic nevus syndrome without prior melanomas, 4 developed their 1st melanoma during prospective follow-up, as compared with 0.03 cases expected. The prospective age-adjusted incidence for melanoma was 14.3/1000 patients with dysplastic nevus per year, with a cumulative melanoma risk (.+-. SE) of 7.2% (.+-. 3.6) at 8 yr. The actuarial probability of melanoma developing in family members with dysplastic nevi was 56.0% (.+-. 10.1) from age 20 to age 59. Dysplastic nevi apparently are clinicaly markers of high risk for, and precursors of, hereditary melanoma.