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Dysplastic nevus syndrome, also known as familial atypical multiple mole–melanoma (FAMMM) syndrome, is an inherited cutaneous condition described in certain families, and characterized by unusual nevi and multiple inherited melanomas.
FAMMM is an abbreviation for Familial Atypical Multiple Mole and Melanoma. People with FAMMM syndrome have the following: One or more first-degree or second-degree relative diagnosed with melanoma at a young age (< 40 years); A large number of naevi (often more than 50), some of which are atypical; Naevi that are dysplastic on histopathology.
Figure 1. The familial atypical multiple mole-melanoma phenotype. (a) Clinically atypical moles frequently associated with FAMMM syndrome. (b) Pedigree of a FAMMM kindred showing multiple early onset cutaneous melanomas (proband and brother) and pancreatic cancer (PANC CA; mother).
Disease definition. Familial atypical multiple mole melanoma (FAMMM) syndrome is an inherited genodermatosis characterized by the presence of multiple melanocytic nevi (often >50) and a family history of melanoma as well as, in a subset of patients, an increased risk of developing pancreatic cancer (see this term) and other malignancies.
Familial atypical multiple mole melanoma (FAMMM) syndrome is an inherited genodermatosis characterized by the presence of multiple melanocytic nevi (often >50) and a family history of melanoma as well as, in a subset of patients, an increased risk of developing pancreatic cancer (see this term) and other malignancies.
More than a century after Norris made his observations, Lynch and Krush 5 described familial atypical multiple mole melanoma (FAMMM) syndrome, which comprised an association between pancreatic cancer (PC), multiple nevi, and melanoma.
30 cze 2019 · Familial atypical multiple mole melanoma (FAMMM) syndrome is an autosomal dominant genodermatosis characterised by multiple melanocytic naevi (often more than 50) and a family history of melanoma. Pathology. Genetics. It is associated with mutations in the CDKN2A gene and shows reduced penetrance and variable expressivity. Associations.
