摘要: |
卡波西型血管内皮瘤(kaposiform hemangioendothelioma, KHE)是一种罕见的具有局部侵袭性的血管肿瘤,好发于婴幼儿,常伴有卡梅现象(Kasabach-Merritt phenomenon, KMP)。KHE目前缺乏基于循证医学的治疗规范。西罗莫司因较好的疗效和安全性有望成为一线用药。本文就目前KHE诊疗进展作一综述。 |
关键词: 卡波西型血管内皮瘤 卡梅现象 西罗莫司 脉管肿瘤 |
DOI: |
分类号:R 732.2 |
基金项目:国家自然科学基金(81572884). |
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Progress in diagnosis and treatment of kaposiform hemangioendothelioma |
YANG Zhang-fu, HU Bo, FU Pei-yao, YU Min-cheng, XU Yang*
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Department of Liver Surgery, Liver Cancer Institute, Zhongshan Hospital, Fudan University, Shanghai 200032
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Abstract: |
Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor with local invasiveness. It is found predominantly in infants and children, and often accompanied by the Kasabach-Merritt phenomenon (KMP). There is no evidence-based standard for KHE treatment yet. The sirolimus is expected to be the first-line drug due to its efficacy and safety. This article reviews the progress of the diagnosis and treatment of KHE. |
Key words: kaposiform hemangioendothelioma Kasabach-Merritt phenomenon sirolimus vascular tumor |