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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 investigate the clinicopathological, immunohistochemical and differential diagnosis of primary liver epithelial hemangioendothelioma (HEHE). Methods The clinical features, histological features and immunohistochemistry of 7 cases of primary hepatocellular hemangioendothelioma diagnosed by biopsy were retrospectively reviewed. The related articles were reviewed with the follow - up results. Results There were 4 males and 3 females in 7 cases. The main clinical manifestations were right upper quadrant pain. Only a few of them were found liver graces at the time of physical examination. Two cases had abnormal liver function. Five cases showed multiple liver lesions. Microscopic epithelial or spindle cells were scattered in a single, cord-like and nested distribution, showing cytoplasmic vacuoles and red blood cells, accompanied by mucoid or fibrous interstitial, immunohistochemical staining of ERG, CD31, CD34, F8 Factors were diffuse (+), CD10 most (+), a few AE1 / AE3, SMA and CK8 / 18 (+). 3 patients died within 1 to 3 months after discharge, 3 patients survived with tumor, and 1 patient lost to follow-up. Conclusion HEHE is a rare malignant tumor of vasculature. The clinical manifestations and examination are nonspecific. HEHE combined with histological morphology and immunohistochemistry, if necessary, confirm the WWTR1-CAMTA1 fusion gene by FISH or RT-PCR.