Adult embryonal sarcoma of the liver:a clinicopathological analysis of four cases
10.13315/j.cnki.cjcep.2024.08.011
- VernacularTitle:成人肝脏胚胎性肉瘤4例临床病理分析
- Author:
Qiwei CAO
1
;
Yazhou ZHANG
;
Qing SUN
Author Information
1. 山东第一医科大学第一附属医院(山东省千佛山医院)病理科/山东省医药卫生临床病理学重点实验室,山东省肺癌研究所,山东省肾脏病研究所,济南 250012
- Keywords:
liver neoplasms;
embryonal sarcoma;
clinicopatho-logical features;
diagnosis;
differential diagnosis
- From:
Chinese Journal of Clinical and Experimental Pathology
2024;40(8):834-838
- CountryChina
- Language:Chinese
-
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.
