单中心儿童肝血管瘤诊治经验的回顾性分析研究  

Diagnoses and treatments of hepatic hemangioma in children:a single-center,retrospective analysis

作　　者：韩立玲 章均[1,2] 朱进 蔡金华[4] 张明满[5] 杨炼[3] 李长春[1] 吕麟亚[1] 王珊[1,2] 魏光辉[2] Han Liling;Zhang Jun;Zhu Jin;Cai Jinhua;Zhang Mingman;Yang Lian;Li Changchun;Lyu Linya;Wang Shan;Wei Guanghui(Ministry of Education's Key Laboratory of Pediatric Development&Disorders,Department of Surgical Oncology,Affiliated Children's Hospital,Chongqing Medical University,Chongqing 400014,China;Ministry of Education's Key Laboratory of Pediatric Development&Disorders,Key Research Laboratory for Pediatric Developmental Diseases of Ministry of Education,National International Science&Technology Cooperation Base for Major Diseases of Pediatric Development,Chongqing Key Laboratory of Pediatrics,400014;Ministry of Education's Key Laboratory of Pediatric Development&Disorders,Department of Pathology,Affiliated Children's Hospital,Chongqing Medical University,Chongqing 400014,China;Ministry of Education's Key Laboratory of Pediatric Development&Disorders,Department of Radiology,Chongqing Medical University,Chongqing 400014,China;Ministry of Education's Key Laboratory of Pediatric Development&Disorders,Department of Hepatobiliary Surgery,Affiliated Children's Hospital,Chongqing Medical University,Chongqing 400014,China)

机构地区：[1]国家儿童健康与疾病临床医学研究中心,重庆医科大学附属儿童医院肿瘤外科,400014 [2]国家儿童健康与疾病临床医学研究中心,儿童发育疾病研究教育部重点实验室,儿童发育重大疾病国家国际科技合作基地,儿科学重庆市重点实验室,400014 [3]国家儿童健康与疾病临床医学研究中心,重庆医科大学病理学教研室400014 [4]国家儿童健康与疾病临床医学研究中心,重庆医科大学附属儿童医院放射科,400014 [5]国家儿童健康与疾病临床医学研究中心,重庆医科大学附属儿童医院肝胆外科,400014

出　　处：《中华小儿外科杂志》2020年第11期982-988,共7页Chinese Journal of Pediatric Surgery

基　　金：重庆市科委社会事业与民生保障科技创新专项(一般,cstc2017shmsA130112);重庆市科委基础科学与前沿技术研究(一般,cstc2017jcyjAX0139)。

摘　　要：目的探讨儿童肝血管瘤的诊断方法及治疗选择,总结不同类型肝血管瘤的治疗经验。方法采用回顾性分析的方法,整群抽取2012年1月至2019年8月重庆医科大学附属儿童医院首次诊治的肝血管瘤患儿的临床资料。将所有入组肝血管瘤患儿按Christison-Lagay分类标准分为局灶型、多发型和弥漫型三组,对各组病史资料、治疗结果进行总结分析。对进行了手术和穿刺活检患儿的病理标本进行Glut-1免疫组织化学染色。结果共145例肝血管瘤患儿纳入本次研究,年龄1 d到7.4岁,包括局灶型126例、多发型12例和弥漫型7例。局灶型患儿中,男77例,女49例;多发型患儿中,男4例,女8例;弥漫型患儿中,男1例,女6例。40例有病理标本患儿的病理检查结果显示,所有多发型和弥漫型肝血管瘤均为婴幼儿血管瘤,Glut-1染色均为阳性;所有局灶型肝血管瘤均为先天性血管瘤,Glut-1染色全部阴性。影像学检查显示,68.25%(86/126)的局灶型肝血管瘤患儿提示有钙化,而多发型和弥漫型肝血管瘤患儿均未见钙化。145例患儿中,有130例(89.66%)通过病史特点和辅助学检查确诊。所有患儿均获得1年以上随访或治疗,目前有116例肝血管瘤患儿的病灶完全消退或治愈,24例病变明显缩小,5例最终死亡。结论局灶型肝血管瘤常常是先天性血管瘤,一般推荐观察随访。而多发型和弥漫型肝血管瘤大多为婴幼儿血管瘤,常规推荐口服普萘洛尔治疗。Objective To explore the diagnoses and treatments of hepatic hemangioma in children and to summarize the management experiences of different types of hepatic hemangioma.Methods From January 2012 to August 2019,clinical data were retrospectively analyzed for 145 initially hospitalized children with hepatic hemangioma at Affiliated Children's Hospital of Chongqing Medical University.There were 77 boys and 49 girls with an age range of 1 day to 7.4 years.According to the Christison-Lagay classification scheme,they were divided into three groups of focal(n=126),multiple(n=12)and diffuse(n=7)types.Immunohistochemical staining of GLUT-1 was performed for the pathological specimens of children undergoing operation and puncture biopsy.Results In focal type,there were 77 boys and 49 girls;4 boys and 8 girls in multiple type;1 boy and 6 girls in diffuse type.The results of pathological examination of 40 children confirmed that all multiple and diffuse hepatic hemangiomas were infantile and the positive rate of GLUT-1 staining was 100%.All focal hepatic hemangiomas were congenital and GLUT-1 staining was negative.And 68.25%(86/126)of children with focal hepatic hemangioma showed calcification while those with multiple/diffuse hepatic hemangioma demonstrated no imaging evidence of calcification.The diagnostic rate based upon medical history and auxiliary examinations was 89.66%(130/145).The follow-up period was over 1 year.Currently 116 cases of hepatic hemangioma completely subsided or became cured,24 cases significantly shrank and 5 cases died.Conclusions Focal hepatic hemangioma is generally congenital.And observations and follow-ups are recommended.Most multiple and diffuse hepatic hemangiomas are infantile.An oral intake of propranolol is recommended.

关 键 词：儿童 肝血管瘤 葡萄糖转运蛋白 普萘洛尔 

分 类 号：R735.7[医药卫生—肿瘤]