原发性腹膜后血管周上皮样细胞瘤合并肾错构瘤及结节性硬化1例报告并文献复习  

Primary retroperitoneal perivascular epithelioid cell tumor coexisting with renal angiomyolipoma and tuberous sclerosis:a case report and literature review

作　　者：吴文铭[1] 邱辉忠[1] 吴斌[1] 林国乐[1] 肖毅[1] 于新明[1] 

机构地区：[1]中国医学科学院北京协和医院基本外科,北京100730

出　　处：《中国实用外科杂志》2010年第1期50-52,共3页Chinese Journal of Practical Surgery

摘　　要：目的探讨原发性腹膜后血管周上皮样细胞瘤(PEComa)的临床病理特点及诊断治疗。方法报告2009年4月北京协和医院基本外科诊治的位于盆腔的原发性腹膜后PEComa1例病人的症状、影像、病理、治疗及随访情况,并结合文献进行分析。结果术前无特异临床症状,为查体发现。术前容易通过影像学手段发现但难以确诊。最终诊断依靠病理学表现和免疫组化。文献报道的原发性腹膜后PEComa非常罕见。结论原发性腹膜后PEComa是一种非常罕见的间叶组织肿瘤,依靠病理学方法诊断。其生物学行为不明确,建议术后长期随访。Objective The first domestic report of primary retroperitoneal perivascular epithelioid cell tumor （ PEComa）. and discuss the clinical pathologic features of the disease. Methods A 29 year-old female patient was admitted in April 2009 in the general surgery department of Peking Union Medical College Hospital and was diagnosed as primary retroperitoneal perivascular epithelioid cell tumor located in the pelvic cavity coexisting with renal angiomyolipoma and tuberous sclerosis. The clinical, imaging, pathology features and treatment of the patient was analyzed retrospectively and related literatures were reviewed. Results The patient had not special symptoms and signs. The imaging methods can easily find the tumor but hard to differentiate it . The final diagnosis depends on the pathologic features and immunohistochemistry techniques. Among present literatures, scarce cases of retroperitoneal PEComa were reported. Conclusions Primary retroperitoneal PEComa is a very rare tumor that arises from mesenchymal tissues. The diagnosis of PEComa mainly depends on the pathological features. Because of the uncertain biologic behavior retroperitoneal PEComa should be followed up for a long term.

关 键 词：腹膜后 血管周上皮样细胞瘤 结节性硬化 

分 类 号：R6[医药卫生—外科学]