摘要
目的探讨多器官发生的上皮样血管内皮瘤(EHE)的临床病理特征、鉴别诊断及预后。方法收集并分析1例多器官发生EHE的临床病理学特征、免疫表型及预后并复习相关文献。结果患者男性,33岁,临床症状主要为腰背疼痛,PET-CT示肝右叶不规则低密度高代谢灶,全身骨多发高代谢溶骨性病灶;肝脏及骨镜下示上皮样肿瘤细胞排列成单个散在细胞、条索状、小巢状,细胞胞质丰富、嗜酸性,胞内可见空泡状,其内可见红细胞,核大深染,核型不规则,间质纤维组织增生伴黏液样变。免疫组化:瘤细胞CD31、CD34、FLi-1、ERG、F8、vimentin均(+),CKpan,EMA,TFE3均(-)。结论 EHE是一种少见的恶性血管源性肿瘤,好发于软组织,部分发生实质脏器,如肺、肝、骨、大脑等,极少数患者可为多脏器同时累及,同时发生于肝脏及骨时,应注意与转移性癌、肝癌伴多发骨转移、上皮样血管肉瘤、上皮样肉瘤等鉴别。
Objective To investigate the clinicopathological features,differential diagnosis and prognosis in multiple organ epithelioid hemangioendothelioma(EHE).Methods The clinicopathological features,immunophenotype and prognosis of 1 multisite EHE case were collected and analyzed.Results The main clinical symptom in a 33-year-old male patient was low back pain.PET-CT examination showed irregular low density and high metabolic foci in the right lobe of the liver.Liver and bone microscopy showed that epithelioid tumor cells were arranged into single scattered cells,clusters and nests,with abundant and eosinophilic cytoplasm,vacuolation containing single red blood cell,large hyperchromatic nuclei,irregular karyotypes,and mesenchymal fibrous tissue hyperplasia with mucinous changes.Immunohistochemical staining showed that the tumor cells were positive for CD31,CD34,FLi-1,ERG,F8 and vimentin,and negative for CKpan,EMA and TFE3.Conclusion EHE is a rare malignancy of vascular origin that occurs in soft tissue,part of the organs,such as lung,liver,bone,brain,etc.In very few patients,multiple organs can be involved simultaneously.Especially,when EHE involves both the liver and bone,we should pay attention to the differential diagnosis from metastatic carcinoma,liver cancer with multiple bone metastases,epithelioid angiosarcoma and epithelioid sarcoma.
作者
黄幼玉
史连国
潘冰川
HUANG You-yu;SHI Lian-guo;PAN Bing-chuan(Department of Pathology,Nanan Hospital,Quanzhou 362300,China;Department of Pathology,the First Hospital Affiliated to Xiamen University,Xiamen 361001,China;Intensive Care Unit,Nanan Hospital,Quanzhou 362300,China)
出处
《诊断病理学杂志》
2021年第7期567-571,共5页
Chinese Journal of Diagnostic Pathology
关键词
多器官发生的上皮样血管内皮瘤
肝脏
骨
临床病理学
诊断
鉴别诊断
Multiple organ epithelioid hemangioendothelioma
liver
Bone
Clinical pathology
Diagnosis
Differential diagnosis
