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中国肝上皮样血管内皮瘤患者的临床及影像学特征

Clinical and radiological characteristics of Chinese patients with hepatic epithelioid hemangioendothelioma.

作者信息

Fan Fei, Yang Xiaoyu, Zhu Bin, Zhang Yongjie

机构信息

Dr. Bin Zhu, Department of Biliary Surgery II,, Eastern Hepatobiliary Surgical Hospital,, Second Military Medical University,, Shanghai 200438, China, T:+862165564166, F:+862165562400,

出版信息

Ann Saudi Med. 2013 Jul-Aug;33(4):334-8. doi: 10.5144/0256-4947.2013.334.

Abstract

BACKGROUND AND OBJECTIVES

To study clinical features, imaging findings, and prognosis of hepatic epithelioid hemangioendothelioma (HEHE) in Eastern Hepatobiliary Surgical Hospital and to improve the level of recognition and preoperative diagnosis of HEHE.

DESIGN AND SETTINGS

This is a retrospective study. The study was conducted at Eastern Hepatobiliary Surgical Hospital in Shanghai, China from 1999 to 2012.

METHODS

Clinical data of 15 pathology-confirmed HEHE patients admitted in our hospital from 1999-2010 were collected and analyzed retrospectively.

RESULTS

Besides 7 cases of abdominal discomfort, this cohort of HEHE patients does not show common typical manifestations. Laboratory examinations found 8 low ALB/GLB cases, 3 mild anemia cases, and 1 high alanine aminotransferase case, but no abnormal a-fetoprotein, carcinoembryonic antigen, or CA19-9 cases. Plain computed tomography (CT) scanning found uneven multiple low-density lesions in most cases, dynamic enhanced CT scanning demonstrated peripheral enhancement in the hepatic arterial HA phase and more peripheral enhancement in the portal vein PV phase, and ring enhancement were detected in the delayed phase. Magnetic resonance imaging (MRI) demonstrated low signal intensity and even lower lesion centers in T1-weighted im.ages, but slightly high signal intensity lesions and higher lesion centers in T2-weighted images. Enhanced MRI scanning showed ring enhancements but no obvious enhanced centers. Lesions were further enhanced in delayed MRI scanning. Prognosis of this cohort: 10 of these 15 patients were alive, including 4 cases with a tumor.

CONCLUSION

Comprehending the clinical and radiological characteristics of HEHE facilitates the level of recognition and preoperative diagnosis of this disease, and promotes surgeons to take active and appropriate surgeries.

摘要

背景与目的

研究东方肝胆外科医院肝上皮样血管内皮瘤(HEHE)的临床特征、影像学表现及预后,以提高对HEHE的认识水平和术前诊断能力。

设计与背景

本研究为回顾性研究。研究于1999年至2012年在中国上海的东方肝胆外科医院开展。

方法

回顾性收集并分析1999年至2010年我院收治的15例经病理确诊的HEHE患者的临床资料。

结果

除7例有腹部不适外,该组HEHE患者无常见典型表现。实验室检查发现8例白蛋白/球蛋白比值降低、3例轻度贫血、1例谷丙转氨酶升高,但甲胎蛋白、癌胚抗原或CA19-9均无异常。平扫计算机断层扫描(CT)大多显示多发不均匀低密度灶,动态增强CT扫描显示肝动脉期周边强化,门静脉期周边强化更明显,延迟期呈环形强化。磁共振成像(MRI)在T1加权像上呈低信号强度,病灶中心信号更低,在T2加权像上呈稍高信号强度,病灶中心信号更高。增强MRI扫描显示环形强化,但无明显强化中心。延迟MRI扫描病灶进一步强化。该组患者的预后:15例患者中10例存活,其中4例有肿瘤。

结论

了解HEHE的临床和影像学特征有助于提高对该病的认识水平和术前诊断能力,并促使外科医生采取积极恰当的手术方式。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1620/6078508/726b4c45e604/asm-4-334f1.jpg

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