小儿卡波西型血管内皮瘤19例临床病理学特点

目的 探讨小儿卡波西型血管内皮瘤的临床病理学特点.方法 收集上海儿童医学中心及新华医院收治的19例卡波西型血管内皮瘤的临床特点及随访结果,应用HE及免疫组织化学染色,观察组织病理学特点,同时统计临床及随访资料.19例患儿中,男11例,女8例;平均年龄19个月,标准差为22.7个月,中位年龄10个月.14例位于皮肤及皮下软组织,3例原发于盆腔,2例原发于骨.临床上多出现局部肿块,少部分因发生凝血异常症状而就诊.实验室检查,8例血小板明显低于正常,7例凝血时间延长及纤维蛋白原降低.结果 本组病理大体标本为不规则实性肿块,无包膜,最大径0.3～1.5 cm.镜下肿瘤形成小叶状结构,边界不清,浸润性生长,小叶由增生的血管和梭形细胞组成,可见新月形和裂隙状血管腔,部分腔内可见微血栓.19例免疫组织化学检测肿瘤细胞表达CD31、CD34和D2-40,不表达Glut-1.19例患儿均经手术切除,其中8例伴有血小板减少的病例,经过对症治疗后再手术切除.随访时间3～75个月,18例术后无复发,1例并发致死性消耗性凝血(kasabach-merritt,KM)现象,患儿死亡.结论 卡波西型血管内皮瘤是少见的交界性儿童血管肿瘤,有特殊的组织学特点,因其可合并KM现象,所以明确诊断对临床意义重大.

Clinicopathological characteristics of pediatric Kaposiform hemangioendothelioma: a report of 19 cases

Objective To explore the clinicopathological characteristics of Kaposiform hemangioendothelioma in children.Methods A total of 19 specimens of Kaposiform hemangioendothelioma were collected from Shanghai Children's Medical Center and Xinhua Hospital in recent years.Their clinical characteristics and follow-up data were analyzed.All cases were observed by hematoxylin & eosin (HE) staining and immunohistochemistry.There were 11 boys and 8 girls with a median age of 10 (19 ± 22.7) months.The lesion sites were skin & subcutaneous soft tissue (n =14),pelvis(n =3) and bone (n =2).Most cases presented with local mass while others were treated for clotting abnormalities.Eight cases showed marked thrombocytopenia and coagulation time prolonged and fibrinogen decreased in another 7 cases.Results The gross specimens were irregular solid mass without capsule with a maximal diameter of 0.3 to 1.5 cm.Microscopic examination revealed unclear marginal lobulated tumors composed of spindle cell,infiltrating surrounding tissue,focally crescent-shaped lumen and microthrombus.Immunohistochemistry was positive for CD31,CD34 and D2-40 and negative for Glut-1.After treatment,8 cases developed thrombocytopenia and resection was performed after supportive measures.During a follow-up period of 3-75 month,18 cases had no recurrence.Only one case complicated by Kasabach-Merritt phenomenon died.Conclusions Pediatric Kaposiform hemangioendothelioma is a rare intermediate vascular tumor with special histological characteristics.Therefore making a definite diagnosis is vital for both pathologists and clinicians.