摘要
目的探讨原发肝脏上皮样血管内皮瘤(HEHE)的临床病理特点,诊断及鉴别诊断。方法回顾性分析9例HEHE患者的临床病理及免疫组化资料。结果男性3例,女性6例,临床表现主要为右上腹不适,其中3例于体检时发现。1例为单发结节,8例为多发。镜下瘤细胞上皮样或树突状,呈小巢状、片状、条索状生长。多数瘤细胞内可见胞质内空泡,内含红细胞似原始血管腔,纤维性间质伴胶原化。免疫组化染色CD31、CD34、FⅧRag、ERG、Fli-1均(+),少数CK、CK7(+)。结论 HEHE是一种恶性血管源性肿瘤,临床表现缺乏特异性,易被误诊或漏诊,诊断主要依据病理形态学及免疫组织化学染色,必要时辅以分子生物学检查。
Objective To investigate the clinicopathological characteristics and immunophenotype of hepatic epithelioid hemangioendothelioma( HEHE). Methods We conducted a retrospective analysis of the clinicopathological data and immunophenotype of 9 patients with HEHE. Results This study was comprised of three males and six females,with the clinical manifestations of right upper abdominal discomfort,weight loss and abdominal distension. Microscopically,round and irregular epithelioid or dendritic tumor cells were arranged in scattered single,nest-like and cord-like patterns. The tumor cells had abundant eosinophilic cytoplasm with prominent vacuoles and single erythrocyte mimicking primitive vascular lumens. The stroma was mainly fibrous tissue hyperplasia with collagenization. Immunohistochemically,tumor cells were strongly positive for CD34,CD31,ERG and factor Ⅷ related antigen( FⅧRAg). Conclusion HEHE is a rare low-grade malignant neoplasm of vascular origin,which is easy to be misdiagnosed. Lack of specificity due to clinical manifestations,the confirmed diagnosis of HEHE mainly depends on pathological examination and immunophenotyping.
作者
丁丽
汪向明
石士奎
李楠
冯振中
郭冰沁
甘怀勇
蔡兆根
DING Li;WANG Xiang-ming;SHI Shi-kui;LI Nan;FENG Zhen-zhong;GUO Bing-xin;GAN huai-yong;CAI Zhao-gen(Department of Pathology,First Affiliated Hospital of Bangbu Medical College,Bangbu 233000,China;Department of Pathology,Wannan Medical College Hospital,Wuhu 241000,China;Department of Radiology,First Affiliated Hospital of Bangbu Medical College,Bangbu 233000,China;Department of Pathology,the Second Hospital of Anhui Medicial University,Hefei 230000,China)
出处
《诊断病理学杂志》
2021年第4期268-271,276,共5页
Chinese Journal of Diagnostic Pathology
关键词
肝脏上皮样血管内皮瘤
血管肿瘤
临床病理学
免疫组化
Hepatic epithelioid hemangioendothelioma
Vascular tumor
Clinicopathology
Immunohistochemistry
