17例肝脏血管周上皮样细胞肿瘤临床分析

A clinical study on 17 patients with liver perivascular epithelioid cell tumors

目的总结肝脏血管周上皮样细胞肿瘤(PEComa)的临床表现,探讨其预后。方法回顾分析深圳市人民医院2002年9月至2020年1月确诊的17例肝脏PEComa患者临床资料,总结其临床表现、影像学特征、病理特点、治疗及预后。结果17例肝脏PEComa患者中女性13例,男性4例,发病年龄为22~54岁,平均发病年龄为34.8岁。无临床症状者占82%(14/17),15例为单发病灶。B超、CT、MRI等术前影像学诊断率较低。术后病理切片分析中,见肿瘤组织多以上皮样细胞为主,其胞质为透明或嗜酸,瘤细胞间有丰富的血管,此外部分病例还可见脂肪组织。免疫组化显示其黑色素瘤抗体HMB45及Melan-A阳性率皆为100%。全部患者经手术切除治疗,随访均未见肿瘤复发、转移及死亡。结论肝脏PEComa好发于青年女性,无特异性临床表现。术前影像学均难以明确诊断,少数患者影像学表现为有脂肪组织可有助于术前鉴别。术后病理结合免疫组化结果能够明确诊断。手术是治疗的主要方法且能够获得良好预后。

Objective To study the clinical manifestations and prognosis of patients with liver perivascular epithelioid cell tumor(PEComa).Methods The clinical manifestations,imaging findings,pathological features,treatment and prognosis of 17 patients with liver PEComa managed in Shenzhen People's Hospital from September 2002 to January 2020 were retrospectively analysed.Results The patient age of onset of liver PEComa was 22 to 54 years(average 34.8 years).There were 13 females and 4 males.82%(14/17)of patients presented with no significant symptoms.15/17 had solitary tumors.The diagnostic rate using preoperative B-ultrasound,CT and MRI was low.Postoperative pathological studies showed the tumor cells were mainly epithelioid cells with hyaline or eosinophilic cytoplasm.There were abundant blood vessels in the tumor tissues.Adipose tissues were also seen.Immunohistochemistry showed both HMB45 and Melan-A positivities to arrive at the diagnosis.All patients were treated with surgical resection.There were no recurrence,metastasis,or death on follow-up.Conclusion Hepatic PEComa occurred more frequently in young women and it had no specific clinical manifestations.Except for a small number of tumors with adipose tissue detected on preoperative imagings,this tumor was difficult to diagnose,or even be suspected on preoperative imagings.Pathological examination combined with immunohistochemistry after surgery established the diagnosis.Surgery resulted in good prognosis.