关于肝上皮样血管内皮瘤治疗的最新信息。

IF 1.1 Q2 MEDICINE, GENERAL & INTERNAL
Kaijian Chu, Zhizheng Li, Wei Tang, Xiaoqing Jiang
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引用次数: 1

摘要

肝上皮样血管内皮瘤(HEHE)是一种罕见的肝脏血管肿瘤,其生物学行为介于血管瘤和血管肉瘤之间。它往往是横跨肝脏的多发或弥漫性囊下病变,但没有特征性的临床表现或影像学发现。在计算机断层扫描和磁共振成像上,这些病变通常表现为低密度且不均匀强化,在某些情况下可能明显出现“光环征”或“棒棒糖征”。HEHE的诊断主要基于病理检查以及不同的免疫组织化学标志物,如CAMTA1、CD31、CD34、CD10、vimentin和factor VIII抗原。目前,HEHE没有标准化的治疗指南,手术(治愈性切除和肝移植)仍然是治疗的主要方式。研究表明肝外转移可能不是切除或移植的禁忌症。包括阿霉素、长春新碱、干扰素-a、5-氟尿嘧啶和沙利度胺在内的全身化疗药物以及vegf相关药物正在研究中,但尚未有药物被批准用于治疗HEHE。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Updated information regarding management of hepatic epithelioid hemangioendothelioma.

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare hepatic vascular tumor with a borderline biological behavior between hemangioma and hemangiosarcoma. It tends to be multiple or diffuse subcapsular lesions across the liver but has no characteristic clinical manifestations or imaging findings. On computed tomography and magnetic resonance imaging, these lesions usually have a hypodense appearance with heterogeneous enhancement and a "halo sign" or "lollipop sign" may be evident in some cases. HEHE is diagnosed mainly based on a pathological examination along with differential immunohistochemical markers such as CAMTA1, CD31, CD34, CD10, vimentin, and factor VIII antigen. Currently, there are no standardized treatment guidelines for HEHE, and surgery (curative resection and liver transplantation) remains the mainstay of treatment. Studies have indicated that extra-hepatic metastasis might not be a contraindication for resection or transplantation. Systemic chemotherapeutic agents including doxorubicin, vincristine, interferon-a, 5-fluorouracil, and thalidomide, as well as VEGF-related agents are being investigated, but no agents have been approved for the treatment of HEHE.

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来源期刊
Intractable & rare diseases research
Intractable & rare diseases research MEDICINE, GENERAL & INTERNAL-
CiteScore
2.10
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发文量
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