CAJ | 학술논문

目的探讨肝血管周上皮样细胞瘤（PEComa）的临床特点。方法回顾性分析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。结果经过积极术前准备，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。结论肝 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。
목적탐토간혈관주상피양세포류（PEComa）적림상특점。방법회고성분석2011년절강대학의학원부속제이의원수치적1례간 PEComa 환자림상자료。환자이첨서지정동의서，부합의학윤리학규정。환자녀，25세，인체검발현간점위성병변입원。체검급실험실검사기본정상。CT 평소시간다개저밀도류원형종괴，변계불청，증강소묘시동맥기종괴명현강화，연지기정등혹초저밀도。MRI 시간병조 T1WI 중등신호，T2WI 초고신호，증강후동맥기종괴명현강화，연지기강화소퇴。림상초보진단위간국조성결절증생。결과경과적겁술전준비，우2011년12월8일재기관삽관전신마취하행간종류절제술。술후병이학검사시종류유류상피세포적다변형세포조성，불함지방세포혹이상혈관。면역조직화학방법검측시종류세포항인흑색소류특이성항체（HMB）45、평활기기동단백(SMA)표체강양성화파형단백、분화군（CD）34양성，병이학진단위간 PEComa。환자회복량호，술후1주출원，정기수방지투고일기미견복발혹전이。결론간 PEComa 시일충겁위한견적병례，림상무특이성표현，학진의뢰우병이학검사。수술절제시치료종류적가고수단，예후교호。
Objective To discuss the clinical characteristics of hepatic perivascularepithelioid cell tumor (PEComa). Methods 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. Results After sufficient preoperative preparation, hepatectomy was performed on the patient under general anesthesia by tracheal intubation on December 8th , 2011. The tumor was observed <br> 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. Conclusions 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.