Abstract
Extramammary Paget's disease (EMPD) is an intraepithelial neoplastic disorder
which is included as a rare malignant condition. However, it sometimes shows
aggressive behavior of local recurrence and coexisting malignancy. We had
experienced nine cases of EMPD involving the scrotum for seven years. Two cases
of them presented metastasis. The first case presented extensive inguinal lymph
node metastasis with underlying adnexal adenocarcinoma one year after wide local
excision. The second case initially presented multiple metastasis to the liver
and in the lymph node. The latter, showing fulminant progression with liver
metastasis, may be only the second case reported in English literature. EMPD is
considered as a malignant neoplasm with aggressive behavior from initial
presentation. Because wide local excision of the lesion alone may be
occasionally insufficient, a careful follow-up must be done to detect recurrence
or internal malignancy.