Orphanet: Familial atypical multiple mole melanoma syndrome

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Familial atypical multiple mole melanoma syndrome

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.


Classification level: Disorder
  • Synonym(s):
    • B-K mole syndrome
    • FAMM-PC syndrome
    • FAMMM syndrome
    • Familial atypical mole syndrome
    • Familial atypical multiple mole melanoma-pancreatic carcinoma syndrome
    • Familial dysplastic nevus syndrome
    • Melanoma-pancreatic cancer syndrome
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: All ages
  • ICD-10: D22.9
  • OMIM: 155600  606719
  • UMLS: C0013403  C0205747  C1838547  C2314896
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information


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