卡波西样血管内皮瘤的研究进展

Recent advances in researches and treatments of kaposiform hemangioendothelioma

卡波西样血管内皮瘤(Kaposiform hemangioendothelioma,KHE)是一种好发于婴幼儿及儿童的、罕见的内皮源性、具有局部侵袭性的脉管肿瘤,表现为弥漫性血管与淋巴管的侵袭性增殖。KHE合并血小板减少、低纤维蛋白血症和凝血因子消耗,称为卡梅现象(Kasabach-Merritt phenomenon,KMP),可致严重并发症甚至死亡。目前,随着大家对KHE发病机制的认识,临床诊疗水平的不断提高,采用手术、动脉栓塞或长春新碱、西罗莫司等药物治疗可使得大多数患儿预后较好;但部分长期合并运动功能障碍与慢性疼痛的患儿,仍是治疗难点。为进一步提高临床医生对KHE的认识,本文拟对其临床特征、主要并发症及危险因素、发病机制和治疗等进行深入阐述,为临床治疗KHE提供经验。

As a rare,endotheliogenic and locally aggressive vascular neoplasm appearing predominantly in infants and toddlers,kaposiform hemangioendothelioma(KHE)is characterized by diffuse and invasive vascular and lymphatic proliferation.It may be associated with thrombocytopenia,hypofibrinemia and coagulation factor consumption,which is also known as Kasabach-Merritt phenomenon(KMP).KMP can cause serious complications and even death.With the continuous progress of pathogenesis,clinical diagnosis and treatments of surgery,embolization and using vincristine and sirolimus,most children have a decent prognosis.However,decreased range of motion and chronic pain are likely to be underappreciated in clinical practice.In this review,recent advances in researches and treatments of KHE were summarized.With a review of the relevant literature,the clinical characteristics,major complications and risk factors of KHE were extensively discussed for its optimal clinical managements.