后腹膜卡波西样血管内皮瘤诊疗分析

Experience in treatment of retroperitoneal kaposiform hemangioendothelioma

目的 通过分析后腹膜卡波西样血管内皮瘤的诊治过程,探讨该类疾病的诊断与治疗方法.方法 复旦大学附属儿科医院外科近年来成功治疗后腹膜卡波西洋血管内皮瘤伴血小板减少患儿2例,回顾性分析2例患儿的病史及诊疗经过.结果 1例患儿肿瘤位于后腹膜,表现为后腹膜巨大占位性病变,伴血小板减少,被误诊为神经母细胞瘤.1例表现为顽固血便,血小板减少,被误诊为出血性肠炎.2例患儿诊断过程复杂,经多科会诊得以确诊.在治疗初期激素治疗有效,但迅速出现耐药和病情反复.采用长春新碱（Vincristine,VCR）治疗后病情得到控制,撤离激素,血小板均恢复至正常范围,肿块消失.结论 后腹膜卡波西样血管内皮瘤临床罕见,最常见的并发症是血小板减少.该病诊断困难,容易误诊.VCR治疗有效,部分患儿可完全缓解.规范化和个体化的治疗非常必要.

Objetive Retroperitoneal kaposiform hemangioendothelioma （KHE） is a rare and locally aggressive vascular tumor that usually occurs in infants.This paper wants to analyze medical records of retroperitoneal kaposiform hemangioendothelioma （KHE） in our hospital,and to share experience and opinions in treatment of such kind of KHE.Methods 2 cases of retroperitoneal KHE with Kasabach merritt Phenomenon （KMP） were diagnosed and treated in Children＇ s Hospital of Fudan University,their medical histories were reviewed.Results Huge retroperitoneal mass or hematochezia with thrombocytopenia in both cases were the main complains.They were misdiagnosed as retroperitoneal neuroblastoma or Necrotizing Enterocolitis （NEC） first,and were confirmed as retroperitoneal KHE after prudent multidisciplineary discussion.Prednisone was effective at the beginning of treatment,but symptoms rebounded shortly after medication withdrew.Vincristine was especially effective on those 2 patients whose symptom were rapidly relieved after 2 dosages of VCR administration.The retroperitoneal masses disappeared finally during follow up in both cases.Conclusion Retroperitoneal KHE with KMP is very rare,and hard to be diagnosed.Understand such kind of disease and multidisciplinary discussion can prevent it from misdiagnosis.VCR is especially effective in patient who resist to predinisone.Standardized and personalize treatment is necessary in treatment of retroperitoneal KHE.