Pediatric Cutaneous Oncology Genodermatoses and Cancer Syndromes

Childhood skin cancer is rare, and its diagnosis should spark the clinical suspicion of an underly in genetic cancer predisposition syndrome. In many of these conditions, the skin is often the first clue to a potential diagnosis. Dermatologists play acrucial role in the detection and treatment of skin cancers; thus, working knowledge of these geno-dermatoses and their extracutaneous clinical associations is necessary to recommend management and improve care. For example, several conditions discussed impact vision. Affected patients typically worry about skin surgery that could impact one or both eyes making them temporarily blind due to bandage placement. Establishing a correct diagnosis in these patients ultimately may involve referral to genetics professional services for testing, and the threshold for referral should be based on heightened clinical suspicions. Advances in genomics have provided information underlying genetic pathways implicated in these conditions and afford insight into potential therapeutic targets. Genetic testing offers the ability to confirm clinical suspicion, and, with accurate diagnosis, the risks of future cancer development can be mitigated with appropriate strategies. While these exciting developments have increased understanding of these conditions, the results of genetic testing can be mired in myriad psychosocial concerns including the potential for discrimination. Early referral to geneticists who can guide appropriate investigations is recommended in these cases to review the benefits of testing and discuss potential ramifications. Additionally, those experienced in clinic genomics can develop comprehensive, personalized management plans for each patient