摘要
Abstract
Purpose To investigate the clinicopathological features,immunophenotype and prognosis of embryonal sarcoma of the liver(ESL)with emphasis on its diagnosis and differenti-al 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 angio-myolipoma,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.Micro-scopically it was composed of myxoid stroma and irregular spin-dle 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 re-section is the key to the treatment for ESL,and preoperative and postoperative combined chemotherapy and radiotherapy should be carried out after surgery.关键词
肝脏肿瘤/未分化胚胎性肉瘤/临床病理特征/诊断/鉴别诊断Key words
liver neoplasms/embryonal sarcoma/clinicopatho-logical features/diagnosis/differential diagnosis分类
医药卫生
