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Table 2 FAMMM Diagnostic criteria for Familial Atypical Multiple Mole Melanoma syndrome [29]

From: CDKN2A founder mutation in pancreatic ductal adenocarcinoma patients without cutaneous features of Familial Atypical Multiple Mole Melanoma (FAMMM) syndrome

1. Malignant melanoma in one or more first- or second-degree relatives

2. High total body nevi count (often >50) including some of which are clinically atypical (asymmetric, raised, color variegation present, of variable sizes)

3. Nevi with certain histologic features on microscopy*

  1. *architectural disorder with asymmetry, subepidermal fibroplasia, and lentiginous melanocytic hyperplasia with spindle or epithelioid melanocytes; variable dermal lymphocyte infiltration and the “shouldering" phenomenon.
  2. All three criteria are needed to make a diagnosis
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Hereditary Cancer in Clinical Practice

ISSN: 1897-4287