Cancer-associated genodermatoses: skin neoplasms as clues to hereditary tumor syndromes.

[cowden syndrome]

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 .