Eccrine sweat gland carcinoma of the scrotum with associated extramammary Paget's disease.

Sweat gland carcinomas are rare tumors which have not previously been reported as arising from the scrotum. We present the first known case of primary eccrine sweat gland carcinoma of the scrotum in association with extramammary Paget's disease (EPD) and review the presentation and management of these tumors. Sweat gland carcinomas frequently coexist with EPD and this association provides insight into the histogenesis of EPD, which is presently unknown. Sweat gland carcinoma should be included in the differential diagnosis of cutaneous scrotal tumors and carefully ruled out pathologically if the diagnosis of EPD alone is made.