摘要目的：探讨儿童脉管异常病变的临床病理特征及国际脉管性疾病研究学会（ ISSVA）新分类的应用价值。方法收集2014年5月至2015年5月117例儿童脉管异常病变的临床特征、组织学形态及免疫组织化学染色结果进行总结分析。结果117例儿童脉管异常病变，年龄出生后18 h至11岁，平均年龄2岁10个月，中位年龄2岁3个月。男73例，女44例，男女比为1．7∶1．0。37例（31．6％）患儿出生即发现皮肤病变。117例病变发生部位：头颈部46例（39．3％）、躯干28例（23．9％）、四肢14例（12．0％）、内脏病变31例（26．5％）。病理学诊断参照ISSVA分类，117例中脉管畸形74例、血管肿瘤43例，二者比值为1．7∶1．0。43例血管肿瘤中以婴儿血管瘤最多，共21例（48．8％），其中增生期17例、退化期4例；先天性血管瘤13例（23．3％），其中快速消退型8例、非消退型5例，发生于肝脏3例；化脓性肉芽肿5例（11．6％）；丛状血管瘤3例（7．0％）；卡波西样血管内皮瘤1例（2．3％）。74例脉管畸形中淋巴管畸形47例（63．5％）；静脉畸形15例（20．2％）；淋巴管静脉畸形11例（14．9％）；动静脉畸形1例（1．4％）。117例脉管异常病变内皮细胞CD31均阳性表达。21例婴儿血管瘤中，增殖期及退化期血管内皮细胞均表达Glut1及CD15，二者在其余血管肿瘤及脉管畸形均阴性。47例淋巴管畸形及11例淋巴管静脉畸形中见D2－40表达；3例丛状血管瘤及1例卡波西样血管内皮瘤中局灶表达D2－40。结论儿童脉管异常包含血管肿瘤及脉管畸形2类不同类型的病变，正确的病理分类在各种脉管异常病变的临床处理中起重要作用。 ISSVA分类标准清晰，指导临床治疗，值得深入学习及应用。

abstractsObjective To study the clinicopathologic features of pediatric vascular anomalies and application of ISSVA classification. Methods The clinical features, histopathologic findings and immunohistochemical results were analyzed in 117 cases of pediatric vascular anomalies encountered during the period from May 2014 to May 2015.Results A total of 117 cases of vascular anomalies were studied. The age of patients ranged from 18 hours after birth to 11 years ( mean age =34 months and median age =27 months) .There were 73 male patients and 44 female patients, with the male-to-female ratio being 1.7∶1.0.Congenital skin lesions were found in 37 cases ( 31.6%) .The common sites of involvement included head and neck region (46 cases, 39.3%), trunk (28 cases, 23.9%), extremities (14 cases, 12.0%) and internal viscera (31 cases, 26.5%).According to the new ISSVA classification, there were 74 cases of vascular malformations and 43 cases of vascular neoplasms ( ratio=1.7∶1.0 ) .The commonest vascular tumor encountered was infantile hemangioma ( 21 cases, 48.8%) , including 17 cases in proliferative phase and 4 cases in involutive phase.Thirteen cases (23.3%) of congenital hemangioma were found, with 8 cases of rapidly involuting congenital hemangioma and 5 cases of non-involutive congenital hemangioma.Three of the congenital hemangioma occurred in liver. There were 5 cases ( 11.6%) of pyogenic granuloma, 3 cases ( 7.0%) of tufted angioma and 1 case ( 2.3%) of Kaposiform hemangioendothelioma.Amongst the 74 cases of vascular malformations encountered, lymphatic malformation was found in 47 cases ( 63.5%) , venous malformation in 15 cases ( 20.2%) , lymphatic-venous malformation in 11 cases (14.9%) and arteriovenous malformation in 1 case (1.4%).All cases of vascular anomalies were all positive for CD31 on immunostaining.Glut1 and CD15 were positive both in proliferative and involutive phases of the 21 cases of infantile hemangioma, while other vascular tumors and vascular malformations were negative.Forty-seven cases of lymphatic malformation and 11 cases of lymphatic-venous malformation showed D2-40 expression.Focal positivity for D2-40 was demonstrated in 3 cases of tufted angioma and 1 case of Kaposiform hemangioendothelioma.Conclusions Vascular anomalies affecting infants and children include tumors and malformations. Accurate histopathologic diagnosis and ISSVA classification of the various types of vascular anomalies play an important role in clinical management.