Abstract: 【Abstract】 Extramammary Paget′s disease （EMPD） is a rare and easily misdiagnosed cutaneous malignancy, which is usually confined to the epidermis and can also infiltrate the dermis in the early stage, and presents with lymph node and distant metastases in the advanced stage. EMPD mostly affects the areas rich in apocrine glands, including the scrotum, penis, and vulva, as well as perianal, inguinal and axillary regions. It typically presents as infiltrative erythema or plaques, some of which are accompanied by scales and erosions. Before the treatment, comprehensively assessing the staging of patients is necessary due to the treatment options depending on the tumor stages. Based on the latest international guidelines and clinical research findings, and taking into account the current status of diagnosis and treatment in China, experts reached a consensus on the first- and second-line treatments and novel treatments according to the staging of EMPD, providing a basis for standardizing the treatment of EMPD.

Key words: Paget disease, extramammary, Neoplasm staging, Antineoplastic protocols, Expert consensus