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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 University 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.

Twenty-two patients with sweat gland carcinoma treated during the past 8 years are reported. They constitute 4.4% of malignant lesions of the skin during the same period. Of these 22 patients, 2 were apocrine gland carcinoma, 15 eccrine carcinoma, 4 hidradenocarcinoma and 1 cylindroma. In 11 patients with distant metastasis, 90.9% had lymphatic metastasis and 36.3% had blood metastasis. Vascular disseminations were mainly to the bone, lung and skin in order of incidence. Sweat gland carcinoma is rare and of low malignancy with evident tendency of recurrence. Prognosis was mainly related to the presence of distant metastasis. It is believed that the enlarged local resection should be given to patients without metastasis. On the other hand, local resection plus regional dissection should be performed on patients with metastasis. Post-operative irradiation may be helpful to prevent distant metastasis.