Cowden's disease: a further indication for prophylactic mastectomy.

Cowden's disease (multiple hamartoma syndrome) is a syndrome involving abnormalities of multiple organ systems. Transmitted in an autosomal dominant pattern, it carries a high frequency of mammary carcinoma in early middle age in affected women. The hyperkeratotic cutaneous and gingival markers of the disease are its principal overt manifestations. Prophylactic bilateral total mastectomy with optional immediate reconstruction is recommended for women Cowden's disease. An illustrative family with the disease is presented in which one affected young woman was found to have invasive mammary carcinoma with regional metastasis at the time of prophylactic mastectomy.