A case of genital Paget's disease with severe dermal invasion and early dissemination

A 65-year-old man was referred to our hospital with a complaint of a scrotal mass which he first noticed 8 months ago. The mass was resected saving genital organs. Pathological diagnosis was extramammary Paget's disease with severe dermal invasion and many nuclear mitoses. The tumor was 15 mm in diameter, and 18 mm in thickness. The bilateral inguinal lymph nodes were dissected and multiple metastases were revealed in the right specimen. Since paraaortic lymph node metastases were detected later 5 cycles of chemotherapy consisting of cyclophosphamide, adriamycin and cisplatin (CAP) followed by radiation therapy were performed. However, only a partial response could be obtained, and multiple brain metastases were revealed on computed tomographic scan. He died 22 months after discovery of this disease. Since extramammary Paget's disease tends to grow slowly and horizontally, cases with such severe dermal invasion and early dissemination as in our case are unusual