Extramammary Paget's disease with aggressive behavior: A report of two cases

Extramammary Paget's disease (EMPD) is an intraepithelial neoplastic disord er which is included as a rare malignant condition. However, it sometimes s hows aggressive behavior of local recurrence and coexisting malignancy. We had experienced nine cases of EMPD involving the scrotum for seven years. T wo cases of them presented metastasis. The first case presented extensive i nguinal lymph node metastasis with underlying adnexal adenocarcinoma one ye ar after wide local excision. The second case initially presented multiple metastasis to the liver and in the lymph node. The latter, showing fulminan t progression with liver metastasis, may be only the second case reported i n English literature. EMPD is considered as a malignant neoplasm with aggre ssive behavior from initial presentation. Because wide local excision of th e lesion alone may be occasionally insufficient, a careful follow-up must b e done to detect recurrence or internal malignancy.