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肝上皮样血管内皮瘤:一种罕见的肝脏血管肿瘤。

Hepatic Epithelioid Hemangioendothelioma: A Rare Vascular Neoplasm of the Liver.

作者信息

Nwabueze Cherechi O, Erhus Efe, Sapkota Abhin, Yeon Su Yeon, Almoghrabi Anas

机构信息

Internal Medicine, John H. Stroger, Jr. Hospital of Cook County, Chicago, USA.

Public Health, George Washington University, Washington, D.C., USA.

出版信息

Cureus. 2025 Jul 28;17(7):e88941. doi: 10.7759/cureus.88941. eCollection 2025 Jul.

DOI:10.7759/cureus.88941
PMID:40895865
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12391529/
Abstract

Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm that can involve multiple organs. Most cases are asymptomatic and detected incidentally, but the clinical course can vary significantly. A 40-year-old man presented with abdominal and chest pain; imaging revealed large hepatic masses along with pulmonary nodules. Histopathological examination confirmed the diagnosis of EHE, with positivity for endothelial markers. Given the extent of the disease, systemic therapy was planned. This case highlights the challenges in diagnosing EHE and underscores the need for an individualized approach to managing this rare tumor.

摘要

上皮样血管内皮瘤(EHE)是一种可累及多个器官的罕见血管肿瘤。大多数病例无症状,为偶然发现,但临床病程差异很大。一名40岁男性出现腹痛和胸痛;影像学检查发现肝脏有巨大肿块以及肺部结节。组织病理学检查确诊为EHE,内皮标志物呈阳性。鉴于疾病范围,计划进行全身治疗。该病例凸显了EHE诊断中的挑战,并强调了对这种罕见肿瘤进行个体化管理的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/27b2/12391529/21ed1f94f77e/cureus-0017-00000088941-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/27b2/12391529/4cb34d2d9f5c/cureus-0017-00000088941-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/27b2/12391529/21ed1f94f77e/cureus-0017-00000088941-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/27b2/12391529/4cb34d2d9f5c/cureus-0017-00000088941-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/27b2/12391529/21ed1f94f77e/cureus-0017-00000088941-i02.jpg

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本文引用的文献

1
ACG Clinical Guideline: Focal Liver Lesions.ACG 临床指南:局灶性肝脏病变。
Am J Gastroenterol. 2024 Jul 1;119(7):1235-1271. doi: 10.14309/ajg.0000000000002857. Epub 2024 Jan 26.
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Epithelioid hemangioendothelioma-its history, clinical features, molecular biology and current therapy.上皮样血管内皮细胞瘤——其历史、临床特征、分子生物学和当前治疗。
Jpn J Clin Oncol. 2024 Jul 7;54(7):739-747. doi: 10.1093/jjco/hyae037.
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Hepatic epithelioid hemangioendothelioma: Clinical characteristics, diagnosis, treatment, and prognosis.肝上皮样血管内皮瘤:临床特征、诊断、治疗及预后
World J Clin Cases. 2022 Jun 16;10(17):5606-5619. doi: 10.12998/wjcc.v10.i17.5606.
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Novel and Specific MRI Features Indicate the Clinical Features of Patients With Rare Hepatic Tumor Epithelioid Hemangioendothelioma.新颖且独特的MRI特征显示罕见肝肿瘤上皮样血管内皮瘤患者的临床特征。
Front Oncol. 2022 Jan 27;12:729177. doi: 10.3389/fonc.2022.729177. eCollection 2022.
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Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts.上皮样血管内皮细胞瘤,一种超罕见的癌症:来自专家社区的共识文件。
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The "Target sign" and the "Lollipop sign" in hepatic epithelioid hemangioendothelioma.肝上皮样血管内皮瘤中的“靶征”和“棒棒糖征”
Abdom Radiol (NY). 2019 Apr;44(4):1617-1620. doi: 10.1007/s00261-018-1820-9.
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Hepatic Epithelioid Hemangioendothelioma.肝上皮样血管内皮细胞瘤。
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Epithelioid hemangioendotheliomas of the liver and lung in children and adolescents.儿童和青少年的肝脏及肺部上皮样血管内皮瘤
Pediatr Blood Cancer. 2017 Dec;64(12). doi: 10.1002/pbc.26675. Epub 2017 Jun 9.
10
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