穿刺活检诊断原发性肝上皮样血管内皮瘤7例临床病理分析  

Primary hepatic epithelioid hemangioendothelioma in core needle biopsy specimens: report of 7cases with review of literature

作　　者：贾旭春[1] 颜临丽[1] 储玮[1] 王哲[1] 

机构地区：[1]第四军医大学西京医院病理科,西安710032

出　　处：《诊断病理学杂志》2015年第11期669-673,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨原发性肝上皮样血管内皮瘤（HEHE）的临床病理特征、免疫组化及鉴别诊断。方法回顾性分析7例通过穿刺活检诊断的原发性肝上皮样血管内皮瘤的临床特征、组织学形态及免疫组化,结合随访结果复习相关文献。结果本组7例中男性4例,女性3例,临床表现主要为右上腹胀痛,少数仅于体检时发现肝占位,2例肝功异常,5例影像学显示肝多发性占位。镜下上皮样或梭形瘤细胞呈单个散在、条索状和巢状分布,可见细胞质内空泡及红细胞,伴有黏液样或纤维样间质,免疫组化染色ERG、CD31、CD34、F8因子均弥漫（＋）,CD10大部（＋）,少数AE1/AE3、SMA和CK8/18（＋）。3例出院后1~3个月内死亡,3例带瘤生存,1例失访。结论 HEHE为少见血管源性恶性肿瘤,临床表现及检查无特异性,需结合组织学形态、免疫组化,必要时用FISH或RT-PCR检测WWTR1-CAMTA1融合基因确诊。Objective To explore the clinicopathological feature,immunohistochemical and differential diagnosis of primary hepatic epithelioid hemangioendothelioma（ HEHE）. Methods The pathological features were investigated in seven cases of HEHE by HE and immunohistochemical staining,with review the correlative literatures. Results The gender of the patients were three females and four males. The symptoms were right upper quadrant abdominal pain. Only two cases showed hypohepatia. Five cases showed multiple liver lesions in computer tomography or MRI. Histologically,the epithelioid tumor cells which contained vacuoles and red blood cells in the cytoplasm were distributed scatteredly or in cords,accompanied by myxoid or fibrous stroma. Immunohistochemically,the tumor cells were diffusely positive for ERG,CD31,CD34 and F8; CD10 staining was positive in the majority cases. Some cases showed AE1 / AE3,SMA,and CK8 /18 positive staining. Three patients died in 1- 3 months after diagnosis,3 patients were alive with disease and 1 was lost in follow-up. Conclusions HEHE is a rare malignant vascular tumor with no significant specificity in clinical manifestation and examination. Its diagnosis needs to combine with histology and immunohistochemistry and rule out other differential diagnosis,when necessary,with FISH or RT-PCR to detect WWTR1-CAMTA1 fusion gene.

关 键 词：肝 上皮样血管内皮瘤 穿刺活检 病理诊断 鉴别诊断 

分 类 号：R735.7[医药卫生—肿瘤]