32例先天性血管瘤临床病理特征及诊治分析  

Management of congenital hemangioma： clinicopathological analysis of 32 cases

作　　者：舒艳[1] 汤炜健 汤宏峰[1] 朱坤[1] 杨敏[1] 赵曼丽[1] 舒强[3] 

机构地区：[1]浙江大学医学院附属儿童医院病理科,杭州310013 [2]浙江大学医学院八年制医学试验班2010级 [3]浙江大学医学院附属儿童医院外科腔镜中心

出　　处：《中华小儿外科杂志》2016年第11期823-827,共5页Chinese Journal of Pediatric Surgery

摘　　要：目的探讨先天性血管瘤（CH）患儿的临床及病理学特征、诊断、鉴别及治疗。方法对32例行手术切除CH患儿的临床资料、病理结果及治疗进行总结。结果患儿男20例，女12例，男女比例为1.6∶1，平均就诊年龄3岁7个月。均为单发肿块，头颈部8例，躯干12例，上肢5例，下肢7例。1例为产前B型超声发现，余31例为患儿出生时发现。25例肿块有逐渐增大表现；3例肿块较出生时有缩小；4例肿块大小无明显变化。1例肿块触诊皮温较高，1例肿物中央可及一窦道，余30例均无局部疼痛、红肿破溃等情况。所有病例术前血常规及凝血谱检查均未见异常。1例患儿曾接受硬化剂注射术，术后肿块无明显缩小。所有病例均行手术完整切除。1例患儿术中出血过多，术后接受输血治疗。结合临床表现后诊断：快速消退型先天性血管瘤（RICH ）3例，余29例为不消退型先天性血管瘤（NICH）。NICH：镜下见瘤组织呈小叶状结构，小叶内微血管内皮细胞核呈＂靴钉样＂向管腔内突出。小叶中央见星形管腔，间质为致密纤维组织，可见淋巴管结构及明显的小动、静脉。RICH：镜下见与NICH相似的小叶状结构，小叶内多量血管闭锁，余血管腔变窄，内皮细胞较扁平，小叶间纤维脂肪组织增多，可见灶性出血坏死、钙化及含铁血黄素沉着。免疫组织化学染色：所有病例均不表达Glut1，均表达CD31、CD34、WT1，D2-40部分表达。随访25例（随访时间3个月至2年1个月），可见残留轻微线样瘢痕，均未见复发。结论CH属良性脉管肿瘤，预后较好。诊断RICH/NICH时需结合临床表现和病理形态。病理上需与脉管畸形、婴儿性血管瘤、卡波西形血管内皮瘤及化脓性肉芽肿相鉴别。Objective To explore the clinicopathological features, diagnosis, differential diagnosis and treatment of congenital hemangioma （CH）. Methods The clinical data, pathological characteristics and treatment of 32 CH cases were analyzed retrospectively. Results There were 20 boys and 12 girls with a boy-to-girl ratio of 1.6：1 and a median age of 43 months. All were unifocal lesions at head ＆ neck region （n = 8）, trunk （n = 12）, upper extremities （n = 5） and lower extremities （n = 7）. One lesion was detected by prenatal ultrasonography and the remainder was present at birth. Twenty-five lesions gradually expanded in size, 3 lesions had slight regression and 4 cases showed no obvious changes. One lesion felt warm on palpation, one with sinus at the center of mass while the remainder had no local pain, swelling or ulceration. All lesions had normal preoperative results o{ blood routine and blood coagulation function. One child received a preoperative injection of sclerosant. However, mass had no regression. All cases underwent complete resection. One child of excessive intraoperative hemorrhage received blood transfusion postoperatively. The clinical types were rapidly involuting congenital hemangioma （RICH, n = 3） and noninvoluting congenital hemangioma （NICH, n = 29）. NICH： Tumor was dominated by lobules of capillaries, separated by dense fibrous tissues containing lymphatic vessels and small arteriovenous structures. Small intralobular vessels were lined by endothelial cells with hobnailed nuclei. The center of lobule was occupied by stelliform vessels. RICH： Tumor was dominated by lohules of capillaries, similar to those seen in NICH, most intralobular vessels were atretic and the remainder diminished. Endothelial cells became flattened. Focal hemorrhagic necrosis, hemosiderosis and calcification were observed, lmmunohistochemistry showed that all cases were negative for Glut1, positive for CD34（32/32）, CD31 （32/32） and WT1 （32/32） and D2-40 was partially positive. Twe

关 键 词：血管瘤 先天性 诊断 鉴别 病理学 临床 

分 类 号：R732.2[医药卫生—肿瘤]