探讨肝血管周上皮样细胞瘤（PEComa）的临床特点。

回顾性分析2011年浙江大学医学院附属第二医院收治的1例肝PEComa患者临床资料。患者已签署知情同意书，符合医学伦理学规定。患者女，25岁，因体检发现肝占位性病变入院。体检及实验室检查基本正常。CT平扫示肝多个低密度类圆形肿块，边界不清，增强扫描示动脉期肿块明显强化，延迟期呈等或稍低密度。MRI示肝病灶T1WI中等信号，T2WI稍高信号，增强后动脉期肿块明显强化，延迟期强化消退。临床初步诊断为肝局灶性结节增生。

经过积极术前准备，于2011年12月8日在气管插管全身麻醉下行肝肿瘤切除术。术后病理学检查示肿瘤由类上皮细胞的多边形细胞组成，不含脂肪细胞或异常血管。免疫组织化学方法检测示肿瘤细胞抗人黑色素瘤特异性抗体（HMB） 45、平滑肌肌动蛋白(SMA)表达强阳性和波形蛋白、分化群（CD）34阳性，病理学诊断为肝PEComa。患者恢复良好，术后1周出院，定期随访至投稿日期未见复发或转移。

肝PEComa是一种极为罕见的病例，临床无特异性表现，确诊依赖于病理学检查。手术切除是治疗肿瘤的可靠手段，预后较好。

To discuss the clinical characteristics of hepatic perivascularepithelioid cell tumor (PEComa).

Clinical data of one patient with hepatic PEComa in the Second Affiliated Hospital, Zhejiang University School of Medicine in 2011 were analyzed retrospectively. The informed consent of the patient was obtained and the ethical committee approval was received. The patient was a 25-year-old female and was admitted in hospital for the physical examination finding of space occupying lesions in the liver. The results of physical and laboratory examinations were normal. Multiple round-like mass of low density with poorly defined borders were observed in the liver by CT scan. Significant enhancement was observed in the arterial phase by enhancement scan, and homo- or hypo-enhancement in the delayed phase. The liver lesions showed intermediated signal intensity on T1WI by MRI and slightly hyperintense on T2WI. Significant enhancement was observed in the arterial phase after enhancement and degraded in the delayed phase. The patient was primarily diagnosed with liver focal nodular hyperplasia clinically.

After sufficient preoperative preparation, hepatectomy was performed on the patient under general anesthesia by tracheal intubation on December 8^th , 2011. The tumor was observed composed of polygonal morphology cells of epithelial cells without lipocytes or abnormal blood vessels by pathological examination. The tumor was observed with strongly positive human melanoma black-45 (HMB-45), smooth muscle actin (SMA), and positive vimentin, cluster of differentiation (CD) 34. The diagnosis of hepatic PEComa was confirmed pathologically. The patient recovered well and was discharged from hospital 1 week after operation. No recurrence or metastasis was observed during the regular follow-up till the submission date.

Hepatic PEComa is extremely rare without specific clinical manifestation. The diagnosis depends on the pathological examination. Surgical resection is an effective method for the tumor with a good prognosis.