肝多发性上皮样血管周细胞肿瘤2例

Hepatic multiple perivascular epithelioid cell neoplasm:Two cases reports

肝脏血管周细胞肿瘤(肝血管平滑肌脂肪瘤),特别是上皮样血管平滑肌脂肪瘤,较为罕见。肝脏多发性上皮样血管平滑肌脂肪瘤临床极为罕见,目前未见系统性报道。本研究总结2例肝脏多发性血管平滑肌脂肪瘤临床病理及组织学表型特点,分析诊断、鉴别诊断、预后资料,并进行文献复习,以期提高临床、影像术前认识。2例多发性肝占位患者,均为女性,年龄分别为37岁和46岁。术前影像学怀疑为肝内转移性肝细胞癌。HE染色显示血管平滑肌脂肪瘤细胞弥漫片状排列,呈上皮样,可见核偏位“鱼眼样”细胞核,肿瘤内可见分化好的厚壁血管及脂肪组织。免疫组织化学片状肿瘤细胞特征性表达黑色素标记A103及HMB45阳性,肝细胞来源标记阴性。2例患者随访48~84个月无复发、转移。肝脏多发性血管平滑肌脂肪瘤预后较好,术前影像学多怀疑为转移性肿瘤,穿刺及免疫组织化学检查可明确诊断,患者预后好。

Multiple hepatic perivascular epithelioid cell neoplasm(MHPEComa)is a really rare type of neoplasm derived from mesenchymal tumors.Hepatic multiple epithelioid angiomyolipoma is extremely rare in clinical practice.Few cases of MHPEComa have been described in the literature to date.A retrospective analysis was performed to investigate the clinicopathological characteristics of 2 patients diagnosed with MHPEComa.Two female patients revealed three and two lesions in the liver respectively,then received surgical resection.Microscopically,detection of epithelioid or spindle-shaped cells with fisheye-like nuclei was suggestive of the disease,and analysis of biomarkers,tumor cells were markedly positive with the melanocytic differentiation marker,Melan-A and HMB45.The hepatic markers were negative.After a follow-up period of 48–84 months,no recurrence was observed.That is often misdiagnosed as hepatocellular carcinoma(HCC),hepatic hemangioma or other liver malignancies.Surgical resection is a definitive curative treatment of hepatic multiple angiomyolipoma when tumour grows or malignancy cannot be excluded.