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Table 3 Familial Atypical Multiple Mole Melanoma (FAMMM) Syndrome Risks and Management. Table listing lifetime cancer risks and management recommendations

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

Cancer Type

Lifetime Risk

Management [28, 29]

Melanoma

58% - 92% by age 80* [6]

From age 10 or in late adolescence as per family history:

- Baseline total body skin examination including scalp, oral mucosa, genital area, and nail, as family members may develop melanoma in their early teens. This screening should also be offered to all first- and some second-degree relatives. Nevi should be examined for ABCDE features of melanoma.**

- Total body photography and sequential digital dermoscopy imaging can be useful tools.

Examination every 3–6 months initially to ensure nevi stability, then annually.

- Thorough total monthly body self-examination should be performed by the patient with assistance from a friend or family member

- Routine sun protective behaviors should be reinforced.

Pancreas

17% - 25% [16, 17]

- Avoid smoking

- lack of evidence-based data to support pancreatic cancer screening

- families should be referred for consideration of clinical research screening programs

  1. *varies with geography
  2. **ABCDE characteristics of the nevus: asymmetric shape, border irregularity, color variegation, diameter greater than 6 mm and elevation or evolution