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[Extramammary Paget's disease of the scrotum with underlying sweat gland adenocarcinoma: a report of six cases with literature review].

Key Laboratory of Oncology in South China, Guangzhou, Guangdong, 510060,P.R. China.
Ai zheng = Aizheng = Chinese journal of cancer 09/2009; 28(8):879-81.
Source: PubMed

ABSTRACT 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.
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.
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.
The primary lesion resection plus uni-inguinal lymphadenectomy is the major treatment for scrotum Paget's disease with underlying sweat gland adenocarcinoma.

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    ABSTRACT: 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 04/2011; 74(4):179-82. · 0.75 Impact Factor

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