成人肝脏胚胎性肉瘤4例临床病理分析

Adult embryonal sarcoma of the liver:a clinicopathological analysis of four cases

目的探讨成人肝脏胚胎性肉瘤(embryonal sarcoma of the liver,ESL)的临床病理特征、诊断、鉴别诊断及预后。方法回顾性分析4例成人ESL的临床影像学资料、组织特征及免疫表型,并进行随访及文献复习。结果4例ESL患者年龄25~49岁,男女比为3∶1;4例均位于肝右叶;CT示巨大类圆形低密度或不规则混杂密度肿块,边界清,其中1例误诊为血管平滑肌脂肪瘤,1例误诊为肝细胞癌,另2例未给出明确诊断。肿物呈实性或囊实性包块,切面鱼肉状,囊内多伴出血、坏死。镜下肿瘤由不规则梭形或星形细胞和疏松黏液样基质构成,散在少量多核巨细胞或瘤巨细胞。瘤细胞核深染,具有高度非典型性及多量核分裂象,特征性表现为可见大小不等的嗜酸性小体,PAS染色阳性。免疫表型:所有病例瘤细胞p53、α1-抗胰蛋白酶(α1-AT)、vimentin等阳性,Ki67增殖指数50%~80%。4例均行手术完整切除,随访4例患者有2例于1年内死亡。结论ESL是成人罕见的高度恶性肿瘤,临床易误诊,确诊依靠于病理学检查,治疗以手术切除肿瘤为首选,手术前、后联合化疗等综合治疗可提高疗效。

Purpose To investigate the clinicopathological features,immunophenotype and prognosis of embryonal sarcoma of the liver(ESL)with emphasis on its diagnosis and differential diagnosis.Methods Four cases of ESL were retrospectively analyzed about the clinicopathological and immunophenotypic profiles with review of the related literatures.Results The 4 ESL patients were 25 to 49 years old,and the male/female ratio was 3∶1.All 4 cases were located in the right lobe of liver.CT showed a large round mass with low density or irregular mixed density with clear boundaries.1 case was misdiagnosed as angiomyolipoma,1 case as hepatocellular carcinoma,and the other 2 cases were not definitively diagnosed.The tumor was a large cystic solid mass with extensive hemorrhage and necrosis.Microscopically it was composed of myxoid stroma and irregular spindle or astroid cells,with multinuclear giant cells or pleomorphic tumor giant cells and characteristic eosinophilic bodies positive for PAS staining.Tumor cells showed hyperchromatic,highly anaplastic nuclei with many mitoses.By immunohistochemistry,the expression of p53,α1-AT and vimentin was detected in all 4 cases,and the proliferation index of Ki67 was 50%-80%.All of the lesions were surgically resected,but two of them died within a year with follow-up.Conclusion ESL is a rare and highly malignant tumor in adults,which is easily misdiagnosed clinically,and the diagnosis depends on pathology.Radical resection is the key to the treatment for ESL,and preoperative and postoperative combined chemotherapy and radiotherapy should be carried out after surgery.