title: Cancer-associated genodermatoses: skin neoplasms as clues to hereditary tumor syndromes.
abstract: Characteristic skin neoplasms are associated with a large number of hereditary tumor syndromes and their knowledge and early detection may facilitate the diagnosis of the underlying malignancies. We will review the clinical and dermatopathological aspects of cutaneous and visceral lesions and the recent progresses in understanding the etiology, pathogenesis and therapies of selected tumor syndromes. The skin neoplasms we chose to consider are multiple neurofibromas in neurofibromatosis, cylindromas and trichoepitheliomas in Broke-Spiegler syndrome, sebaceous tumors and keratoacanthomas in Muir-Torre syndrome, Gardner fibromas in Gardner syndrome, multiple basal cell carcinomas in nevoid basal cell carcinoma (Gorlin) syndrome, multiple tricholemmomas in Cowden syndrome, multiple fibrofolliculomas in Birt-Hogg-Dubé syndrome and multiple leiomyomas in hereditary leiomyomatosis and renal cell cancer. Hereditary cancers have distinct biological and clinical features as compared to their sporadic counterparts; for this reason, we are now able to experiment new treatment approaches involving not only tumor detection and prevention, but also tailored therapeutic strategies focusing on the peculiar druggable molecular targets.
authors: Department of Clinical and Diagnostic Medicine and Public Health, University Hospital of Modena and Reggio Emilia, Italy. giovanni.ponti@unimore.it
authors: Giovanni
authors: G
authors: Ponti
authors: Giovanni
authors: G
authors: Pellacani
authors: Stefania
authors: S
authors: Seidenari
authors: Annamaria
authors: A
authors: Pollio
authors: Umberto
authors: U
authors: Muscatello
authors: Aldo
authors: A
authors: Tomasi
mesh_terms: D006801:Humans; D009386:Neoplastic Syndromes, Hereditary / Q000175:diagnosis / Q000209:etiology*; D012878:Skin Neoplasms / Q000175:diagnosis / Q000209:etiology*