Synonyms
B-K mole syndrome; Familial atypical mole and melanoma syndrome (FAM-M); Familial atypical multiple mole and melanoma syndrome (FAMMM); Hereditary dysplastic nevus syndrome
Definition
Familial dysplastic nevus syndrome (familial DNS) is a genetically heterogeneous hereditary form of DNS. Familial melanoma – the occurrence of melanoma in at least two blood relatives of the first or second degree in one side of the family – is often associated with familial DNS. The descriptive term “familial atypical multiple mole and melanoma syndrome” (FAMMM) emphasizes this association; the term “atypical mole” is used instead of “dysplastic nevus.”
FAMMM and familial DNS are often used interchangeably, the preference of use of one over the other being subject to the same controversy as the use of the term “dysplastic nevus.” Strictly, familial melanoma is not a requisite for the diagnosis of familial DNS; however, the diagnostic criteria for familial DNS have not been agreed upon. Nowadays,...
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References and Further Reading
Bressac-de Paillerets, B., & Demenais, F. (2006). Familial cutaneous melanoma. In P. E. LeBoit, G. Burg, D. Weedon, & A. Sarasain (Eds.), World Health Organization Classification of Tumors. Pathology and genetics of skin tumours (pp. 279–281). Lyon: IARC Press.
Diagnosis and Treatment of Early Melanoma. NIH Consensus Statement Online (1992). Jan 27–29 (cited 2010-08-23);10(1), 1–26.
Hussein, M. R., & Wood, G. S. (2002). Molecular aspects of melanocytic dysplastic nevi. Journal of Molecular Diagnostics, 4(2), 1–4.
Kraemer, K. H., & Greene, M. H. (1985). Dysplastic nevus syndrome. Familial and sporadic precursors of cutaneous melanoma. Dermatologic Clinics, 3(2), 225–237.
Tsao, H., et al. (2003). The transformation rate of moles (Melanocytic Nevi) into cutaneous melanoma: A population-based estimate. Archives of Dermatology, 139(3), 282–288.
Tucker, M. A., et al. (2002). A natural history of melanomas and dysplastic nevi: An atlas of lesions in melanoma-prone families. Cancer, 94(12), 3192–3209.
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Li-Ning, E.M., Torres-Cabala, C.A. (2016). Familial Dysplastic Nevus Syndrome. In: Massi, D. (eds) Dermatopathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-30006-1_2812
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DOI: https://doi.org/10.1007/978-3-319-30006-1_2812
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