Atypical Mole Syndrome

Aka: Atypical Mole Syndrome, Dysplastic Nevus Syndrome, Familial Melanoma, Familial Cutaneous Melanoma, FAM-M Syndrome, B-K Mole Syndrome, FAMMM Syndrome

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II. Epidemiology

  1. Families predisposed to Atypical Nevi and Melanoma
  2. Family History of relatives with Melanoma onset at a young age
  3. Prevalence: 32000 in United States with Atypical Mole Syndrome
    1. Accounts for ~5% of moles diagnosed in the United States
  4. Atypical Nevi in those with Atypical Mole Syndrome carry significant risk of malignant transformation
    1. Melanoma 10 year risk: 10.7% (>17 fold higher risk over those without syndrome)
    2. Melanoma lifetime risk: Approaches 100%

III. Pathophysiology

  1. Superficial spreading Melanoma is more common in these families
  2. Autosomal Dominant inheritance

IV. Diagnosis: Atypical Mole Syndrome

  1. Malignant Melanoma in at least one first degree or second degree relative
  2. Large number of melanocytic nevi (typically over 50), variable in size and some with atypia
  3. Histology demonstrates Atypical Nevi

V. Associated Conditions

  1. No association with other cancers

VI. References

  1. Habif (2003) Clinical Dermatology, 4th ed.. Mosby, p. 773-813
  2. Marghoob (1994) Arch Dermatol 130(8): 993-8 [PubMed]

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