Role of Heredity in Melanoma Susceptibility A Primer for the Practicing Surgeon James F. Abdo, MD a , Aayush Sharma b , Rohit Sharma, MD a, * INTRODUCTION Through clinical practice and in laboratories around the world, information accumula- tion in the area of melanoma genetics is being rapidly translated to actionable knowl- edge in the clinic. Advent of molecular and genomic evaluation techniques further our understanding of the underlying mechanisms of the complex interplay between hered- itary factors and the environment. This information will help clinicians identify high risk a Department of Surgery-Oncology Division, Marshfield Medical Center- Marshfield, Marsh- field, WI, USA; b Ohio State University, Columbus, OH, USA * Corresponding author. Marshfied Clinic, Department of General Surgery, 1000 N Oak Avenue, Marshfield, WI 54449. E-mail address: sharma.rohit@marshfieldclinic.org KEYWORDS  Melanoma  Hereditary melanoma  Familial melanoma  CDKN2A  FAMMM  Cancer syndromes KEY POINTS  Heredity should be considered an independent risk factor for melanoma, that may modify environmental risk factors.  Familial melanoma is a poorly defined term. It is most commonly understood as a syn- drome based on specific phenotypic characteristics including multiple family members with melanoma, early age of onset, multiple primary melanomas, and coexistence of other primary tumors.  Families with known germline CDKN2A mutations generally have multiple family members with melanoma, early age of onset, multiple primary melanomas in an individual, and coexistence of other primary tumors. However, germline mutations in CDKN2A are un- common even in the presence of these phenotypic traits and family history.  The probability of an individual with familial melanoma having an associated identifiable gene mutation will vary depending upon criteria used to define familial melanoma and geographic location.  Genetic counseling is recommended for patients thought to be at increased risk for mel- anoma with either a characteristic phenotype or strong family history of melanoma to assist providers in medical decision making. Surg Clin N Am - (2019) -–- https://doi.org/10.1016/j.suc.2019.09.006 surgical.theclinics.com 0039-6109/19/ª 2019 Elsevier Inc. All rights reserved.