肝上皮样血管内皮瘤1例并文献复习

目的提高对肝上皮样血管内皮瘤的认识。方法结合我院诊治的一例肝上皮样血管内皮瘤的患者资料和文献复习,探讨该病的临床特点、诊断、治疗及预后等。结果肝上皮样血管内皮瘤病因尚不明确,最常见的临床表现是右上腹疼痛、肝肿大和体重减轻,MRI或CT特点为:病灶对比增强扫描大于3 cm的病灶增强后有向心性强化倾向,而小于3 cm的病灶呈环形强化,门脉期及延迟期持续环形强化,肝内静脉主干及分支终止于肿瘤的边缘,形成"棒棒糖征"。病理学检查主要特征是具有细胞内血管腔的上皮样瘤细胞并呈血管内皮标志物染色阳性。肝上皮样血管内皮瘤的治疗以手术切除和肝移植为主,预后介于良、恶性肿瘤之间。结论肝上皮样血管内皮瘤发病率低、临床表现复杂,易误诊,临床诊治过程中应予以重视。

Hepatic epithelioid hemangioendothelioma:a case report and review of the literature

Objective To discribe the clinical feature of hepatic epithelioid hemangioendothelioma.Method With a case report and review of the related literatures,the etiology,clinical manifestations,diagnosis,management and prognosis of hepatic epithelioid hemangioendothelioma were discribed.Results The causes of hepatic epithelioid hemangioendothelioma remain unclear,The most common clinical symptom was right upper quadrant pain,hepatomegaly and weight loss.The characteristics of MRI or CT were that enhanced scan lesions with more than 3 cm were enhanced centripetally;however,the lesion with less than 3 cm showed ring-enhancement,and persisted in portal and delayed phase.The intrahepatic venous trunk and branches terminated in tumor margin,forming "lollypop symptom".Pathologic characteristics showed vascular cavity epithelioid tumor cells and vascular endothelial markers showing positive staining.The treatments of hepatic epithelioid hemangioendothelioma were mainly surgical resection and liver transplantation.The prognosis are between benign and malignant tumors.Conclusions The hepatic epithelioid hemangioendothelioma has a low incidence,the clinical symptoms are complicated,easily misdiagnosed,thus it should be attended during the process of clinical diagnosis.