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Atypical Mole Syndrome
Aka: Atypical Mole Syndrome, Dysplastic Nevus Syndrome, Familial Melanoma, Familial Cutaneous Melanoma, FAM-M Syndrome, B-K Mole Syndrome, FAMMM Syndrome
- See Also
- Atypical Nevus
- Melanoma
- Epidemiology
- Families predisposed to Atypical Nevi and Melanoma
- Family History of relatives with Melanoma onset at a young age
- Prevalence: 32000 in United States with Atypical Mole Syndrome
- Accounts for ~5% of moles diagnosed in the United States
- Atypical Nevi in those with Atypical Mole Syndrome carry significant risk of malignant transformation
- Melanoma 10 year risk: 10.7% (>17 fold higher risk over those without syndrome)
- Melanoma lifetime risk: Approaches 100%
- Pathophysiology
- Superficial spreading Melanoma is more common in these families
- Autosomal Dominant inheritance
- Diagnosis: Atypical Mole Syndrome
- Malignant Melanoma in at least one first degree or second degree relative
- Large number of melanocytic nevi (typically over 50), variable in size and some with atypia
- Histology demonstrates Atypical Nevi
- Associated Conditions
- No association with other cancers
- References
- Habif (2003) Clinical Dermatology, 4th ed.. Mosby, p. 773-813
- Marghoob (1994) Arch Dermatol 130(8): 993-8 [PubMed]