阴囊Paget病合并汗腺癌6例报道及临床分析

Extramammary Paget’s disease of the scrotum with underlying sweat gland adenocarcinoma: a report of six cases with literature review

背景与目的:阴囊Paget病合并汗腺癌极为少见,本文总结阴囊Paget病合并汗腺癌的临床病理特点及其治疗情况。方法:回顾性分析1964-2004年我院收治的阴囊Paget病合并汗腺癌患者6例,复习文献并分析其典型表现、诊断及治疗方法。结果:阴囊Paget病合并汗腺癌典型表现以湿疹样皮肤改变为主。本组所有患者接受病灶切除并患侧腹股沟淋巴结清扫术,1例行腹直肌带蒂皮瓣转移术。术后15、26、38月各死亡1例,3例术后随访48,50,55个月,未见复发。结论:病灶手术切除及患侧腹股沟淋巴清扫是治疗阴囊Paget病合并汗腺癌的主要方法。

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.