Hepatic epithelioid hemangioendothelioma: imaging features and experience in clinical diagnosis and treatment

宁周雨; 陈其文; 朱晓燕

doi:10.19401/j.cnki.1007-3639.2016.12.007

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Hepatic epithelioid hemangioendothelioma: imaging features and experience in clinical diagnosis and treatment

更新时间：2025-12-31

- Hepatic epithelioid hemangioendothelioma: imaging features and experience in clinical diagnosis and treatment
- China Oncology Vol. 26, Issue 12, Pages: 1004-1010(2016)
- 作者机构：
  
  复旦大学附属肿瘤医院中西医结合科，复旦大学上海医学院肿瘤学系,上海,200032
- 作者简介：
- 基金信息：
- DOI：10.19401/j.cnki.1007-3639.2016.12.007
  CLC：
- Published Online：23 January 2017，
  
  Published：23 January 2016
- 稿件说明：
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宁周雨,陈其文,朱晓燕,等. 肝脏上皮样血管内皮瘤的影像特点及临床诊治体会[J]. 中国癌症杂志, 2016, 26(12): 1004-1010.

宁周雨, 陈其文, 朱晓燕. Hepatic epithelioid hemangioendothelioma: imaging features and experience in clinical diagnosis and treatment[J]. China Oncology, 2016, 26(12): 1004-1010.
宁周雨,陈其文,朱晓燕,等. 肝脏上皮样血管内皮瘤的影像特点及临床诊治体会[J]. 中国癌症杂志, 2016, 26(12): 1004-1010. DOI： 10.19401/j.cnki.1007-3639.2016.12.007.

宁周雨, 陈其文, 朱晓燕. Hepatic epithelioid hemangioendothelioma: imaging features and experience in clinical diagnosis and treatment[J]. China Oncology, 2016, 26(12): 1004-1010. DOI： 10.19401/j.cnki.1007-3639.2016.12.007.

摘要

背景与目的：肝脏上皮样血管内皮瘤(hepatic epithelioid haemangioendothelioma，HEHE)是一种极为罕见的血管源性肿瘤，临床及影像诊断很容易误诊。本研究通过总结HEHE的临床及影像特点，为今后HEHE的临床诊治提供重要参考。方法：回顾分析复旦大学附属肿瘤医院中西医结合科住院的5例经病理证实的HEHE患者的临床表现、影像特征、病理特点及临床治疗的疗效。结果：5例患者中，男性2例，女性3例，年龄范围26~65岁，平均45.6岁，2例肝内多中心发生，3例全身多组织、多中心发生。B超为等低回声，病灶内无明显血流信号。CT平扫为等低密度，MR平扫为低T1高T2信号，内密度及信号不均匀，CT/MR增强扫描时病灶呈“慢进慢出”式明显、环状强化特点。PET/CT为明显高FDG摄取，且呈延迟显像的特点。DSA造影下见肿瘤血管纤细，肝动脉化疗栓塞(transcatheter arterial chemoembolization，TACE)术后病灶碘油沉积不理想。显微镜下肿瘤细胞呈上皮样分化并出现细胞质内血管腔形成，免疫组织化学法检测5例均呈CD31和CD34阳性。5例患者均存活，其中2例经TACE联合高强度聚焦超声(high intensity focused ultrasound，HIFU)和(或)射频消融(radiofrequency ablation，RFA)肿瘤局部控制效果显著。结论：HEHE具有特征性的临床表现及影像学特征，目前对于全身多组织、多中心发生的晚期患者，TACE联合HIFU和(或)RFA可能是最有效的方法。

Abstract

Background and purpose: Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare

vascularly original tumor

and would be misdiagnosed easily in the clinical and imaging characteristics. This study aimed to investigate the imaging features of HEHE and our experience in clinical diagnosis and treatment

and to provide important reference for the diagnosis and treatment of HEHE in the future. Methods: This study retrospectively analyzed clinical manifestations

imaging features

pathological characteristics and treatment effects of 5 HEHE cases confirmed by pathology at Department of Integrative Cancer

Fudan University Shanghai Cancer Center. Results: The ratio of male to female patients was 2 to 3. HEHE predominantly occurred in middle-aged female patients whose ages range from 26 to 65 (mean=45.6). Imaging features of HEHE included multifocal hepatic disease (n=2)

systemic multi-center multi-tissue occurrence (n=3). The ultrasound images showed isoechoic or hypoechoic lesions with no obvious blood flow signal in the lesions. CT plain scan showed isointensity or hypointensity

while MR unenhanced with hypointense T1 signal and hyperintense T2 signal. The density or signal was uneven. Contrast-enhanced CT and MR images showed “slow in slow out”

obvious and variable degrees of peripheral rim enhancement. High FDG uptake showed delayed imaging characteristics (PET/CT). DSA angiography showed the tumor blood vessels were slim. After transcatheter arterial chemoembolization (TACE) surgery

lipiodol deposition within the lesion was not ideal. Under the microscope

tumor cells showed epithelial differentiation; angiogenesis was also visible. Immunohistochemistry staining showed CD31 and CD34 positive in all the 5 cases. Two cases treated with TACE combined with high intensity focused ultrasound (HIFU) and/or radiofrequency ablation (RFA) had good result. Conclusion: The clinical and radiological characteristics of HEHE are distinctive. Currently

for patients with systemic multi-organizational multi-center lesions

TACE combined with HIFU and/or RFA might be the most effective treatment method.

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