Genodermatoses with Cutaneous Tumors and Internal Malignancies

The genodermatoses reflect a diverse group of disorders characterized by cutaneous findings associated with an underlying genetic defect. A subset of these conditions are known to confer a susceptibility to the development of cutaneous and internal tumors. Powerful genetic technologies are continuously advancing our understanding of the molecular basis of these familial cancer syndromes. This article reviews those genodermatoses with cutaneous and extracutaneous tumors, focusing on recent insight into their clinical presentation and molecular etiology, because familiarity with these may facilitate early diagnosis and minimize morbidity from their associated malignancies.