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 a
nd management of these tumors. Sweat gland carcinomas frequently coexi
st with EPD and this association provides insight into the histogenesi
s 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 m
ade. (C) 1997, Elsevier Science Inc. All rights reserved.