卡波西样血管内皮瘤117例临床分析

Clinical Features of 117 Cases of Kaposiform Hemangioendothelioma

目的总结分析卡波西样血管内皮瘤(Kaposiform hemangioendothelioma,KHE)患者的临床特点及治疗经验。方法选择并分析2008年1月-2018年12月在四川大学华西医院就诊的117例KHE患者的临床资料,结合相关文献,回顾性总结分析其临床特征、并发症、治疗及预后等特点。结果共入组117例KHE患者,男64例、女53例,比例约为1.20∶1,患者年龄1 d^5岁,平均年龄2.74个月,年龄<1岁的患者105例(89.74%)。伴有KMP(Kasabach-Merritt phenomenon,KMP)者83例,主要临床表现为皮肤损害、皮下或骨关节肌肉的浸润性肿物、伴有运动受限、急性心衰、胸腔积液、心包积液或凝血出血相关疾病等并发症。病灶直径范围2~26 cm,平均直径为9.04 cm。提示年龄、病灶大小与患者是否发生KMP有关(P均<0.05)。采用西罗莫司、泼尼松、长春新碱等药物治疗55例,手术治疗25例,联合治疗32例,未治疗行观察随访5例。转归:临床治疗有效103例(88.03%),9例(7.69%)因急性心衰、呼吸衰竭、弥散性血管内凝血等并发症死亡。结论绝大多数KHE患者在1岁以前发病,并且发生KMP风险更高。KHE临床表现主要与各种并发症有关,早期明确诊断和及时治疗对减少并发症的发生、改善预后十分重要。

Objective The purpose of this study was to summarize and analyze the clinical characteristics and treatment experience of Kaposiform hemangioendothelioma(KHE).Methods We retrospectively reviewed medical records of 117 KHE patients from January 2008 to December 2018.The clinical characteristics,complications and prognosis of patients were analyzed.Results In total,117 KHE patients were enrolled,including 64 males and 53 females,with a ratio of 1.20∶1.The age ranged from 1 day to 5 years,and the average age is 2.74 months.A total of 105 patients(89.74%) were under the age of one.There were 83 patients with Kasabach-Merritt phenomenon(KMP).The major clinical manifestations included skin lesions,subcutaneous or joint muscle-invasive lesions,decreased range of motion,acute heart failure,pleural effusion,pericardial effusion and blood coagulation-related diseases.The size of the lesion ranged from 2 to 26 cm,with an average diameter of 9.04 cm.Age and lesion size were related to the occurrence of KMP(P<0.05).Treatment:55 cases were treated with monotherapy with sirolimus,hormone,vincristine or other drugs;25 cases received surgical treatment;32 cases received combination therapy,and 5 cases received no treatment.Clinical treatment was effective in 103 cases(88.03%).Nine cases(7.69%) died due to acute heart failure,respiratory failure,diffuse intravascular coagulation and other complications. Conclusion The majority of KHE patients had symptoms before the age of one and have a higher risk of KMP.The clinical manifestations of KHE are related to various complications.Early diagnosis and timely treatment are crucial for the treatment of KHE,the reduction of complications and the improvement of prognosis.