Three cases of metastatic extramammary Paget’s disease successfully treated with etoposide

Background: Extramammary Paget's disease (EMPD) is a malignant skin tumor that commonly occurs in the vulva, axilla, and perianal region, and it often presents as an intraepidermal lesion. However, when it invades the dermis, leading to lymph node metastasis and distant metastasis, the prognosis becomes poor. Due to its rarity, no anti-cancer drugs are covered by insurance in Japan, but a few case reports revealed the effectiveness of chemotherapy by docetaxel, etoposide, cisplatin and 5-fluorouracil for advanced EMPD.