A case of extramammary Paget's disease of 62 year-old male is reported. The patient noted an erythematous plaque on his scrotum about five years ago. Although the lesion was diagnosed as a malignant tumor at that time in another hospital, he neglected adequate therapy. By the time he visited our hospital with numbness in the toes and gait disturbance, the lesion had progressed to an infiltrative erythema with tumor on his scrotum, penis and pubis. Furthermore, bilateral inguinal and iliac lymph node metastases and multiple bone metastases were found. Wide excision and inguinal lymphadenectomy were performed and a metastatic lesion in the backbone was also removed as a palliative measure for the symptoms of numbness in the toes and the gait disturbance. Five courses of postoperative chemotherapy with actinomycin D and vincristine led to disappearance of lymph node metastases in the pelvis, prevented recurrence of the tumor and decreased the serum CEA level to almost within normal range. The chemotherapy, however, had to be stopped because of side-effects such as high fever and general fatigue.
Hypercalcemia due to bone metastases became marked. He died in 9 months after initiation of the treatment.