肝脏血管周上皮样细胞瘤2例临床诊治分析

Clinical analysis of hepatic perivascular epithelioid cell neoplasm:A report of two cases

目的探索肝脏血管周上皮样细胞瘤(PEComa)的疾病特点和诊治方案。方法收集暨南大学第一附属医院2018年8月至2019年11月2例肝脏血管周上皮样细胞瘤患者的临床资料,分析其临床表现、影像学特点、诊断及治疗策略、病理特点、预后。结果2例肝脏PEComa患者均为女性,没有典型的临床症状及体征,AFP、CEA、CA⁃199等肿瘤指标均为阴性,B超、上腹MRI等影像学检查结果考虑为肝癌,治疗上采取腹腔镜肝肿物切除术,术后复查未见肿瘤复发及远处转移征象。结论肝脏PEcoma是少见的间叶源性肿瘤,以良性为主,术前诊断误诊率高,组织学及免疫组化学检查是确诊的主要方法。主要治疗措施是手术切除及长期密切随访。

Objective To investigate the clinical features of hepatic perivascular epithelioid cell carcinoma(PEComa)and the experiments in the diagnosis and treatment to hepatic PEComa.Methods The clinical data of two patients with hepatic PEComa at The First Affiliated Hospital of Jinan University from August 2018 to November 2019 were collected.The clinical manifestations,imaging features,diagnostic and therapeutic strategies,pathologic features,prognosis were analyzed.Results The two patients with hepatic PEComa were female,with no specific clinical symptoms and signs.The tumor indicators such as AFP,CEA and ca⁃199 were negative,and the imaging diagnosis was liver cancer.Two patients received a surgical resection and no recurrence or metastasis was observed.Conclusion Hepatic PEcoma is a rare mesenchymal tumor,which is mainly benign and difficult to diagnose before surgery.Histological and immunochemical examination are the main methods of diagnosis.The main treatment measures are surgical resection and long⁃term close follow⁃up.