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肝血管肉瘤与上皮样血管内皮瘤的临床及磁共振成像特征比较

Comparison of clinical and MRI features of hepatic angiosarcoma and epithelioid hemangioendothelioma.

作者信息

Kim Hae Young, Hong Sun, Heo Subin, Song In Hye, Kim Jihun, Yoo Youngeun, Kang Hyo Jeong, Park Seong Ho, Lee Seung Soo, Kim So Yeon

机构信息

Department of Radiology, Asan Medical Center, Seoul, Republic of Korea.

Department of Radiology, Ansan Hospital, Korea University College of Medicine, Ansan, Republic of Korea.

出版信息

Abdom Radiol (NY). 2025 Feb;50(2):619-632. doi: 10.1007/s00261-024-04513-3. Epub 2024 Aug 13.

Abstract

OBJECTIVE

Differentiating hepatic epithelioid hemangioendothelioma (EHE) and angiosarcoma (AS), the two most common vascular tumors in the liver, is important due to disparities in their prognosis and treatment. We aimed to compare clinical and MRI features of the two tumors.

METHODS

This retrospective study included patients with pathologically-confirmed AS or EHE who underwent MRI using gadoxetate disodium between 2008 and 2023. Two radiologists independently reviewed MR images. Wilcoxon rank sum and Fisher's exact tests were used to compare clinical and imaging features. Overall survival was compared using restricted mean survival time at 3 years.

RESULTS

32 patients with AS (18 women [56.3%]; median age, 68 years) and 38 with EHE (24 women [63.2%]; 51 years) were included. Patients with AS were generally older (81.3% ≥ 60 years; P < 0.001), had more frequent laboratory abnormalities (P ≤ 0.018), and poorer overall survival (11.2 vs. 31.8 months; P < 0.001) than those with EHE. On MRI, a large dominant mass accompanied by smaller nodules (14/32, 43.8%), often with ill-defined margins (15/32, 46.9%) was prevalent in AS; compared with nodules of similar sizes (24/38, 63.2%; P = 0.015) with well-defined margin (30/38, 78.9%; P = 0.002) in EHE. Cirrhotic appearance of the liver was more frequent in AS (62.5%, P < 0.001), along with decreased parenchymal enhancement on hepatobiliary phase (31.3%, P < 0.001) and ascites (37.5%, P = 0.010). AS frequently presented with avid enhancement of bizarrely-shaped foci, with a centrifugal enhancement pattern. In comparison, targetoid appearance was characteristic of EHE (78.9% on T2-weighted, 54.1% on diffusion-weighted, 65.8% on multiphase images) (P ≤ 0.002), with enhancement degree typically lower than that of the aorta. On hepatobiliary phase, all the AS exhibited hypointensity, while 39.5% of EHE showed targetoid appearance (P < 0.001).

CONCLUSION

Patients aged ≥ 60 years presenting with laboratory abnormalities, typically with a large dominant mass accompanied by smaller nodules, exhibiting avid, bizarre, and centrifugal enhancement-particularly in the cirrhotic-appearing liver-suggests the likelihood of AS over EHE.

摘要

目的

肝上皮样血管内皮瘤(EHE)和血管肉瘤(AS)是肝脏中两种最常见的血管肿瘤,由于它们在预后和治疗方面存在差异,因此对二者进行鉴别诊断很重要。我们旨在比较这两种肿瘤的临床和MRI特征。

方法

这项回顾性研究纳入了2008年至2023年间接受钆塞酸二钠增强MRI检查且病理确诊为AS或EHE的患者。两名放射科医生独立审查MR图像。采用Wilcoxon秩和检验和Fisher精确检验比较临床和影像特征。使用3年时的受限平均生存时间比较总生存期。

结果

纳入32例AS患者(18例女性[56.3%];中位年龄68岁)和38例EHE患者(24例女性[63.2%];51岁)。与EHE患者相比,AS患者通常年龄更大(81.3%≥60岁;P<0.001),实验室异常更常见(P≤0.018),总生存期更差(11.2个月对31.8个月;P<0.001)。在MRI上,AS常见大的优势肿块伴小的结节(14/32,43.8%),边缘常不清(15/32,46.9%);而EHE中类似大小的结节更常见(24/38,63.2%;P=0.015),边缘清晰(30/38,78.9%;P=0.002)。AS患者肝脏更常表现为肝硬化外观(62.5%,P<0.001),肝胆期实质强化降低(31.3%,P<0.001)以及腹水(37.5%,P=0.010)。AS常表现为奇异形状病灶的快速强化,呈离心性强化模式。相比之下,靶样表现是EHE的特征(T2加权像上78.9%,扩散加权像上54.1%,多期图像上65.8%)(P≤0.002),强化程度通常低于主动脉。在肝胆期,所有AS均表现为低信号,而39.5%的EHE表现为靶样外观(P<0.001)。

结论

年龄≥60岁、有实验室异常、通常有大的优势肿块伴小的结节、表现为快速、奇异和离心性强化——尤其是在有肝硬化外观的肝脏中——提示更可能是AS而非EHE。

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