Melanoma predisposition is hereditary in 10% of cases. Large international research projects have been set up to unravel many of the remaining issues concerning familial melanoma, which include the following:

• Identifying the causative gene defect in the majority of families.

• Determining precise risk estimates for melanoma and other cancers, especially pancreatic cancer, in mutation carriers.

• Attributing the degree and causes of the increased risk of melanoma in nonmutation carriers of mutationpositive families.

• Elucidating the underlying causes of the nevus phenotype and its risk implications.

• Assessing the efficacy of surveillance programs for melanoma and other cancers.

At present, the clinical management of familial melanoma should be restricted to recognition of the disease and subsequent regular skin examinations of high-risk individuals.

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