卡波西样血管内皮瘤与丛状血管瘤临床对比研究  

A comparative study on the clinical features of kaposiform hemangioendothelioma and tufted angioma

作　　者：代诗懿 张学鹏 周江元 邱桐 杨开颖 陈思源[2] 吉毅[1] Dai Shiyi;Zhang Xuepeng;Zhou Jiangyuan;Qiu Tong;Yang Kaiying;Chen Siyuan;Ji Yi(Department of Pediatric Surgery,West China Hospital,Sichuan University,Chengdu 610041,China;Department of Critical Care Medicine,West China Hospital,Sichuan University,Chengdu 610041,China)

机构地区：[1]四川大学华西医院小儿外科,成都610041 [2]四川大学华西医院重症医学科,成都610041

出　　处：《中华整形外科杂志》2022年第3期284-290,共7页Chinese Journal of Plastic Surgery

基　　金：国家自然科学基金(81401606、81400862);四川大学优秀青年学者基金(2015SU04A15);四川省科技计划重点研发项目(2019YFS0322);四川大学华西医院学科卓越发展1·3·5工程临床研究孵化项目(2019HXFH056、2020HXFH048、ZYJC21060)。

摘　　要：目的比较卡波西样血管内皮瘤(KHE)与丛状血管瘤(TA)的临床特征异同。方法回顾性分析2016年8月至2020年6月在四川大学华西医院诊治的KHE患者和TA患者的临床资料。比较两种疾病在性别及发病年龄、临床特征、并发症、组织病理学、影像学表现、治疗方式及预后等指标上的异同。两组定量资料因不符合正态分布,采用Mann-Whitney U检验进行分析,定性分类资料采用χ2检验或Fisher精确检验进行分析,以P<0.05为差异有统计学意义。结果共纳入217例患者,其中KHE患者183例,TA患者34例。KHE与TA在男女比例(χ^(2)=0.44,P=0.510)、发病年龄(U=2757.00,Z=-1.09,P=0.278)及发病部位(χ^(2)=3.64,P=0.162)方面,差异无统计学意义。KHE以混合型最常见,达63.39%(116/183),TA以浅表型最常见,达88.24%(30/34)。KHE患者病灶直径为6.30(4.40,9.70)cm,大于TA患者的2.95(2.05,4.03)cm,两者差异具有统计学意义(U=967.50,Z=-6.38,P<0.001)。KHE引起骨骼肌受累、血小板减少症与严重纤维蛋白原减少症可能性更大。KHE主要累及真皮、皮下组织甚至深部肌肉,表现为浸润性肿物,磁共振成像(MRI)在T2相上呈团块状弥漫性高信号,而TA往往为仅累及皮下脂肪层的浅表病灶,MRI显示高信号局限于皮下脂肪层。KHE总治疗有效率为85.79%,总药物治疗有效率为85.32%,均低于TA的91.18%和95.65%。结论与TA相比,KHE病变范围更大,侵袭性更高,发生并发症的风险更高,治疗难度更大,治疗方案更复杂,治愈有效率较TA低。Objective To investigate the similarities and differences in clinical manifestations,lesion features,treatment options and prognosis in patients with kaposiform hemangioendothelioma(KHE)and tufted angioma(TA).Methods The clinical data of KHE patients and TA patients diagnosed and treated in West China Hospital of Sichuan University from August 2016 to June 2020 were retrospectively analyzed.The similarities and differences in gender,the age of onset,clinical features,complications,histopathology,imaging manifestations,treatment and prognosis of the two diseases were compared.The χ^(2) test or Fisher’s exact test were used to analyze the qualitative data.The Mann-Whitney U test was used to analyze the quantitative data of the two groups that did not conform to the normal distribution.P<0.05 was considered to be statistically significant.Results A total of 217 patients were included,including 183 patients with KHE and 34 patients with TA.There was no significant difference between KHE and TA in male to female ratio(χ^(2)=0.44,P=0.510),the age of onset(U=2757.00,Z=-1.09,P=0.278)and the sites of lesion(χ^(2)=3.64,P=0.162).The mixed type of KHE was the most common,reaching 63.39%(116/183),while the superficial type of TA was the most common,reaching 88.24%(30/34).The lesion diameter of KHE patients was 6.30(4.40,9.70)cm,which was larger than that of TA patients 2.95(2.05,4.03)cm,and the difference was statistically significant(U=967.50,Z=-6.38,P<0.001).KHE is more likely to involve skeletal muscle,and cause thrombocytopenia and severe fibrinogenopenia.KHE mainly involved the dermis,subcutaneous tissue and even deep muscles,and manifested as an infiltrative mass.Magnetic resonance imaging(MRI)showed mass diffuse with high signal on T2 phase.TA was often a superficial lesion that only involved the subcutaneous fat layer and was lumpy.MRI showed that the high signal was confined to the subcutaneous fat layer.The total effective rate of KHE[KHE(85.79%)vs.TA(91.18%)]and the total effective rate of drug therapy[KHE(85.

关 键 词：血管内皮瘤 KASABACH-MERRITT综合征 丛状血管瘤 

分 类 号：R732.2[医药卫生—肿瘤]