Extramammary Pageťs disease of the scrotum with underlying sweat gland adenocarcinoma: A report of six cases with literature review

Abstract

Background and Objective: Extramammary Paget's disease (EMPD) of the scrotum with sweat gland adenocarcinoma is a rare malignant tumor. This study was to summarize the clinicopathologic characteristics of scrotum Paget's disease with underlying sweat gland adenocarcinoma, and analyze the treatment outcome. Methods: Clinical data of six scrotum Paget's disease patients with sweat gland adenocarcinoma, treated in Sun Yat-sen Univercity Cancer Center from 1964 to 2004, were analyzed with literature review. Results: The typical manifestation of scrotum Paget's disease with sweat gland adenocarcinoma was eczematoid-like skin changes. All patients underwent primary lesion resection plus uni-inguinal lymphadenectomy, one patient underwent rectus abdominis pedicle flap transplantation. Three patients died of tumor at 15, 26, 38 months after operation, respectively. Other three patients were followed up for 48, 50, 55 months, respectively, and were alive without tumor. Conclusion: The primary lesion resection plus uni-inguinal lymphadenectomy is the major treatment for scrotum Paget's disease with underlying sweat gland adenocarcinoma.