5例肝脏血管周围上皮样细胞肿瘤临床特点分析  

Clinical features of patients with hepatic perivascular epithelioid cell tumor: An analysis of 5 cases

作　　者：张芸 郁义星[1] 赵卫峰[1] Zhang Yun;Yu Yixing;Zhao Weifeng(Department of Infectious Diseases,First Affiliated Hospital,Soochow University,Suzhou 215006,Jiangsu Province,China)

机构地区：[1]苏州大学附属第一医院感染病科,江苏省苏州市215006

出　　处：《实用肝脏病杂志》2023年第5期722-725,共4页Journal of Practical Hepatology

摘　　要：目的 分析5例血管周围上皮样细胞肿瘤(PEComa)患者的临床、影像学和病理学资料,总结其临床特征,以提高对其诊断和鉴别诊断能力。方法 回顾性分析5例肝占位病变患者的临床资料,行钆塞酸二钠增强MRI检查,经手术后组织病理学或肝脏穿刺细胞学检查诊断为PEComa。所有患者接受肝叶切除术。结果 2例血清总胆红素、2例丙氨酸氨基转移酶和1例天冬氨酸氨基转移酶轻度升高,血清甲胎蛋白、白蛋白和凝血酶原时间均正常;血清HBsAg阳性3例;5个病灶均为单发,其中肝右叶4例,肝左叶1例;呈分叶状或类圆形;4个病灶边界清晰,1个病灶边界模糊;瘤体1.2×1.3 cm~6.9×9.0 cm;MRI检查发现肝内病灶T1WI呈低信号5例,T2WI呈高信号4例,动脉期强化5例,其中3例病灶内见瘤内血管或早期引流静脉,门脉期呈低信号4例,等信号1例,肝胆特异期呈低信号5例,DWI呈高信号5例,同反相位见信号衰减3例;5例均接受肝部分切除术,术后随访10~263个月,患者情况良好,均未见复发。结论 PEComa在临床和影像学表现上与肝癌相似,但预后良好。在无肝炎、肝硬化背景患者出现类似肝癌的征象时,应注意鉴别,尤其是存在动脉期均匀强化伴有瘤内血管或早期静脉引流、门脉期肿瘤边缘呈高信号、部分存在脂肪征象时,应考虑到PEComa可能,结合血清肿瘤指标可帮助诊断。Objective This paper summarized the clinical features of 5 patients with hepatic perivascular epithelioid cell tumor(PEComa)in order to improve the clinician’s ability for diagnosis and differentiation of intrahepatic occupying lesions.Methods The clinical materials of 5 patients with PEComa were retrospectively analyzed with the emphasis on imaging features.All patients underwent gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid(Gd-EOB-DTPA)MRI scan and hepatectomy.Results Serum bilirubin levels increased in 2 cases,ALT increased in 2 cases and AST increased in 1 case with normal serum AFP,albumin and prothrombin time in all 5 patients;serum HBsAg positive in 3 cases;all the patients had a single intrahepatic lesion,with in the right lobe in 4 cases and in the left in 1 cases;the tumor sizes were 1.2×1.3 cm-6.9×9.0 cm shaped lobulated or elliptical,with clear edges in 4 lesions and obscure edge in 1 lesion;the MRI T1WI showed low signal in 5 cases,T2WI showed high signal in 4 cases,intensified in artery phase in 5 cases,presenting as low signals in portal phase in 4 case,equal signal in 1 case,and DWI showed high signal in 5 cases;all 5 patients got along well as followed-up for 10-263 months.Conclusion The PEComa could have similar clinical and imaging manifestations to primary liver cancer,but might have a good prognosis.The clinicians should paid attention to differentiation when the patients have no background of viral hepatitis or cirrhosis,especially when there is a uniform enhancement in arterial phase accompanied by intratumoral vessels or early venous drainage,high signal at tumor edge in portal vein phase,and some signs of fat infiltration.

关 键 词：肝脏血管周围上皮样细胞肿瘤 磁共振成像 临床特征 

分 类 号：R735.7[医药卫生—肿瘤]