肾上腺血管周上皮样细胞瘤1例报告并文献复习  

Adrenal perivascular epithelioid cell tumor:a case report and literature review

作　　者：袁平成[1] 郭刚[2] 马鑫[2] 陈小刚[1] 张青汉[1] 

机构地区：[1]黄石市中心医院,湖北理工学院附属医院泌尿外科,湖北黄石435000 [2]解放军总医院泌尿外科,北京100853

出　　处：《临床泌尿外科杂志》2015年第7期617-620,共4页Journal of Clinical Urology

摘　　要：目的:探讨原发性肾上腺血管周上皮样细胞瘤(PEComa)的临床病理特点及诊断治疗方法。方法:报告2014年11月中国人民解放军总医院泌尿外科诊治原发性肾上腺PEComa 1例患者的临床特征、影像学特点、病理学结果、治疗及随访情况,并结合文献进行分析。结果:文献报道的肾上腺PEComa非常罕见,该病术前多无特异临床症状,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结果,治疗首选手术切除,术后须长期随访。结论:原发性肾上腺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： To discuss the clinical pathologic features of primary adrenal perivaseular epithelioid cell tumor （PEComa）. Method： A 34-year-old female was admitted in November 2014 in the department of urology of general hospital of PLA and was diagnosed as primary adrenal PEComa. The clinical, imaging, and pathologic fea- tures and treatment of the patient was analyzed retrospectively and related literature was reviewed. Result.. Among present literature, scarce cases of adrenal PEComa were reported. The patient had no special symptoms and signs. The imaging methods could easily find the tumor, but it was hard to differentiate it. The final diagnosis depended on the pathologic features and immunohistochemistry techniques. Surgical resection was the preferred treatment and a long-term follow-up plan was also needed after surgery. Conclusion： Primary adrenal PEComa is a very rare tumor that arises from rnesenchymal tissues. Its diagnosis mainly depends on the pathological features. The pa- tient should be followed up for a long period because of the uncertain biologic behavior of primary adrenal PECo- ma.

关 键 词：肾上腺肿瘤 血管周上皮样细胞瘤 治疗 

分 类 号：R736.6[医药卫生—肿瘤]