卡波西型血管内皮瘤13例临床分析  

Clinical analysis on 13 cases of Kaposiform hemangioendothelioma

作　　者：高鹏飞[1] 舒曼[2] 谢钧韬[1] 谢起根 高文宗[1] 周李[1] 

机构地区：[1]中山大学附属第一医院小儿外科,广州510080 [2]中山大学附属第一医院病理科,广州510080

出　　处：《中华实用儿科临床杂志》2017年第11期841-844,共4页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的 探讨卡波西型血管内皮瘤（Kaposiform hemangioendothelioma，KHE）的诊断与治疗，加深对KHE及卡-梅现象（Kasabach－Merritt phenomenon，KMP）的认识，探索KHE的最佳治疗方案。方法 收集2008年1月至2016年8月于中山大学附属第一医院就诊、经手术或穿刺病理确诊13例KHE患儿的临床诊疗资料，并进行回顾性分析。结果 13例患儿，男7例，女6例；年龄0.2-10.0岁，中位年龄1.0岁，婴幼儿占84.6%（11/13例）；浸润深部组织者占76.9%（10/13例）；并发KMP占23.1%（3/13例），年龄均〈1岁；伴血管瘤或淋巴管瘤者占15.4%（2/13例）。影像学检查有助于分析病变部位、范围和浸润深度，镜下病理可见梭形或卵圆形细胞呈结节状浸润生长。手术治疗尽量做到完整切除；应用长春新碱每周0.5 mg/m^2；普萘洛尔1 mg/（kg·d）。13例患儿预后不尽相同，随访3个月-9年，带瘤生存者占41.7%（5/12例）。结论 KHE多见于婴幼儿，临床表现多样，复发率高，影像学检查有助于诊疗，但需病理明确诊断，可伴血管淋巴管瘤生长。伴有KMP时，应首先予以纠正，治疗要综合考虑，制订个体化的治疗方案，并进行长期追踪。Objective To explore the diagnosis and treatment of Kaposiform hemangioendothelioma （ KHE）, deepen the understanding of KHE and Kasabach - Merritt phenomenon （KMP） , and discuss the optimal treatment for KHE. Methods From January 2008 to August 2016,13 cases of KHE were confirmed by surgery or biopsy pathology and admitted to the First Affiliated Hospital of Sun Yat - Sen University, and the clinical diagnosis and treatment were analyzed retrospectively. Results There were 13 patients（7 males and 6 females） with a median age of 1.0 years （0. 2 - 10.0 years） ,and 84.6% （ 11/13 cases） were infants and young children,76.9% （ 10/13 cases） involved with deep tissue,23. 1% （3/13 cases） were associated with KMP and they were younger than 1 year old,and 15.4% （2/13 cases） coexisted with hemangioma or lymphangioma. The location, extent and infiltration depth of the lesion were observed by imaging examinations and histopathology showed nodule shaped spindle tumor cells. Radical resection was considered if possible. Dose of Vincristine （0.5 mg/m^2 weekly） and Propranolol [ 1 mg/（ kg·d） ] were administered. The prognosis was different in thirteen cases undergoing different treatments. After 3 months to 9 years follow - up, 41.7% （5/12 cases ） survived after tumor treatment. Conclusions KHE happens mostly in infants and young children,with varying clinical manifestations and a high recurrence rate. The diagnosis of KHE is based on histological examination, computed tomography and magnetic resonance imaging while it still need explicit pathological diagnosis is needed. KHE may be accompanied by hemangioma or lymphangioma. Prognosis is affected by many factors and the comprehensive treatment is required. KMP should be remedied preferentially, individual treatment protocol and long term follow - up are necessary.

关 键 词：卡波西型血管内皮瘤 卡-梅现象 诊断 治疗 分析 

分 类 号：R732.2[医药卫生—肿瘤]