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上皮样血管内皮细胞瘤是一种危险、容易被忽视且几乎无法临床诊断的疾病:病例报告。

Epithelioid Hemangioendothelioma as a Dangerous, Easy to Miss, and Nearly Impossible to Clinically Diagnose Condition: Case Report.

机构信息

Department of Dermatology, Campbell University, Sampson Regional Center, Clinton, NC, United States.

Campbell University School of Osteopathic Medicine, Lillington, NC, United States.

出版信息

JMIR Dermatol. 2024 Aug 28;7:e52493. doi: 10.2196/52493.

DOI:10.2196/52493
PMID:39196615
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11391149/
Abstract

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with metastatic potential. EHE can have single- or multiorgan involvement, with presentations ranging from asymptomatic disease to pain and systemic symptoms. The extremely heterogeneous clinical presentation and disease progression complicates EHE diagnosis and management. We present the case of a 24-year-old woman with two periauricular erythematous papules, leading to the discovery of metastatic EHE through routine biopsy, despite a noncontributory medical history. Histology revealed the dermal proliferation of epithelioid cells and vacuoles containing red blood cells. Immunohistochemistry markers consistent with EHE solidified the diagnosis. Although extremely rare, prompt diagnosis of EHE is essential for informed decision-making and favorable outcomes. Key clinical and histopathological findings are highlighted to aid dermatologists in diagnosing and managing this uncommon condition.

摘要

上皮样血管内皮细胞瘤(EHE)是一种罕见的具有转移潜能的血管肿瘤。EHE 可单发或多器官受累,表现从无症状疾病到疼痛和全身症状不等。极其异质性的临床表现和疾病进展使 EHE 的诊断和管理变得复杂。我们报告了一例 24 岁女性患者,其双侧耳前有红斑性丘疹,尽管病史无明显提示,但通过常规活检发现转移性 EHE。组织学显示真皮内上皮样细胞和含有红细胞的空泡增生。免疫组织化学标志物与 EHE 一致,从而明确了诊断。尽管极其罕见,但及时诊断 EHE 对于做出明智的决策和获得良好的预后至关重要。强调了关键的临床和组织病理学发现,以帮助皮肤科医生诊断和管理这种罕见疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d82/11391149/f1072cae23e9/derma_v7i1e52493_fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d82/11391149/434a02115610/derma_v7i1e52493_fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d82/11391149/5ddf0bbf9aa0/derma_v7i1e52493_fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d82/11391149/f1072cae23e9/derma_v7i1e52493_fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d82/11391149/434a02115610/derma_v7i1e52493_fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d82/11391149/5ddf0bbf9aa0/derma_v7i1e52493_fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d82/11391149/f1072cae23e9/derma_v7i1e52493_fig3.jpg

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本文引用的文献

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The heterogeneity of Epithelioid Hemangioendothelioma (EHE): A case series and review of the literature with emphasis on treatment options.上皮样血管内皮瘤(EHE)的异质性:病例系列及文献综述,重点关注治疗选择
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