卡波西样血管内皮瘤/丛状血管瘤发病机制  

Pathogenesis of Kaposiform Hemangioendothelioma and Tufted Angioma

作　　者：于鲁 尉莉[1] 徐子刚[1] 张斌[1] 韩晓锋[1] 孙玉娟[1] 刘元香 王忱[1] 邱磊 修冰玉 何瑞 马琳[1] 李丽[1] YU Lu;WEI Li;XU Zigang;ZHANG Bin;HAN Xiaofeng;SUN Yujuan;LIU Yuanxiang;WANG Chen;QIU Lei;XIU Bingyu;HE Rui;MA Lin;LI Li(Department of Dermatology,Beijing Children fs Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,China)

机构地区：[1]国家儿童医学中心首都医科大学附属北京儿童医院皮肤科,北京100045

出　　处：《中国皮肤性病学杂志》2022年第3期250-255,共6页The Chinese Journal of Dermatovenereology

基　　金：北京市医院管理局儿科专项重点项目(XTZD20180502)。

摘　　要：卡波西样血管内皮瘤(kaposiform hemangioendothelioma,KHE)/丛状血管瘤(tufted angioma,TA)为血管内皮细胞肿瘤,具有局部侵袭性,部分伴发卡梅现象(Kasabach-Merritt phenomenon,KMP),是一种具有潜在死亡风险的危重症。目前已报道的KHE/TA的发病机制主要涉及遗传学改变、起源于向淋巴管和血管方向分化的肿瘤干细胞、mTOR通路异常等,KHE/TA向KMP的转化主要涉及血小板捕获。本文将对KHE/TA的临床、病理特点以及目前已经研究报道的发病机制进行阐述。Kaposiform hemangioendothelioma(KHE)/tufted angioma(TA)is a locally aggressive vascular tumor that is partly associated with occurrence of Kasabach-Merritt phenomenon(KMP)and is a critical illness with potential risk of death.The reported pathogenesis of KHE/TA involves genetic changes,tumor stem cells that differentiate into the lymphatic and blood vessels,abnormal mTOR signal pathway,and the transformation from KHE/TA to KMP mainly involves platelet capture.This article reviews the clinical and pathological characteristics of KHE/TA and the pathogenesis of KHE/TA that has been reported.

关 键 词：卡波西样血管内皮瘤 丛状血管瘤 卡梅现象 发病机制 雷帕霉素 

分 类 号：R739.5[医药卫生—肿瘤]