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Extramammary Paget's disease of the scrotum with sweat gland adenocarcinoma is a rare malignant tumor. This study aims to summarize the clinicopathologic characteristics related to the prognosis of scrotal Paget's disease with underlying sweat gland adenocarcinoma. Clinical datum of four patients with scrotal Paget's disease with sweat gland carcinoma, treated in Beijing Chao-Yang Hospital from 2002 to 2009 was analyzed, and a literature review was conducted. The typical manifestation of scrotal Paget's disease with sweat gland carcinoma was eczematoid-like skin lesions. All patients underwent primary lesion resection plus inguinal lymphadenectomy. Three patients had inguinal lymph metastasis. One of them developed distant metastases in bone and bone marrow and died of metastatic carcinoma. The dead patient had higher serum carcinoma embryonic antigen (CEA) level, Her-2 overexpression and shorter disease course than the other patients. The other patients were observed for at least 3 years, and lived without tumor. Scrotal Paget's disease with sweat gland adenocarcinoma may be prone to inguinal lymph node and bone metastasis. Serum CEA level, Her-2 overexpression, dermis and lymphovascular invasion may be associated with the prognosis of scrotal Paget's disease with sweat gland adenocarcinoma. The primary lesion resection plus inguinal lymphadenectomy is the major treatment for scrotal Paget's disease with sweat gland adenocarcinoma. The effect of combination chemotherapy in the treatment of metastatic extramammary Paget's disease remains to be proven by prolonged follow-up and wide experience.

journal of the chinese medical association

Clinicopathologic characteristics of extramammary Paget’s disease of the scrotum associated with sweat gland adenocarcinoma—a clinical retrospective study