Familial Atypical Multiple Mole Melanoma Syndrome

Familial atypical multiple mole melanoma (FAMMM) syndrome is an autosomal dominant genodermatosis characterized by multiple melanocytic nevi, usually more than 50, and a family history of melanoma (Figure 1 and Figure 2). It is associated with mutations in the CDKN2A gene and shows reduced penetrance and variable expressivity. Some FAMMM kindreds show an increased risk for the development of pancreatic cancer and possibly other malignancies (1-6).