[Hereditary cutaneous tumor syndromes]

Skin tumors can manifest solitarily and sporadically but can also be multiple and familial. Beside the skin, hereditary cutaneous tumor syndromes also affect extracutaneous organs and are clinically and genetically heterogeneous. Taking the medical history, a clinical examination and dermatopathological characterization of the respective neoplasia will help the dermatologist to reach a diagnosis at an early stage. Subsequently, this diagnosis can be unambiguously confirmed by molecular genetic analysis. Here, we provide an overview and update on selected hereditary cutaneous tumor syndromes.