首页|儿童肾母细胞瘤相关的易感综合征筛查与相应的肾母细胞瘤治疗进展

儿童肾母细胞瘤相关的易感综合征筛查与相应的肾母细胞瘤治疗进展

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肾母细胞瘤是儿童最常见的恶性肿瘤之一,约占儿童肿瘤的7%.其中10%~17%的肾母细胞瘤与肿瘤易感综合征(cancer predisposition syndrome,CPS)有关,此类肾母细胞瘤发病更早,恶性度及双侧发病率更高.目前非易感型综合征的肾母细胞瘤的治疗生存率可达90%以上,治疗方案包括手术及联合放化疗,而易感型综合征肾母细胞瘤治愈成功率较低,为了保留肾单位、改善预后,其治疗也各不相同.与肾母细胞瘤相关的CPS有WAGR综合征、Denys-Drash综合征和Beckwith-Wiedemann综合征等.目前国内医师缺少对肾母细胞瘤相关的易感综合征预防及诊断的认识,本文通过回顾相关文献,对肾母细胞瘤相关的易感综合征的筛查和相应的肾母细胞瘤治疗方法进行综述.
Screening for susceptibility syndromes associated with Wilms tumor in children and recent thera-peutic advances
Wilms tumor(WT)is one of the most common malignancies in children.It accounts for around 7%of pediatric tumors.And 10%-17%of WTs are associated with cancer predisposition syndrome(CPS)with an earlier onset,a greater malignancy and a higher incidence.Currently survival rate of WT in non-susceptible syndrome is over 90%.Treatment options include surgery plus chemoradiotherapy.Success rate of WT in susceptible syndrome is low and treatments and outcomes vary.The associated susceptibility syndromes include WT-Aniridia-Genitourinary Anomalies-Mental Retardation(WAGR),Denys-Drash and Beckwith-Wiedemann.Currently domestic physicians lack a basic understanding of treating and preventing WT-related syndromes.This review focused upon screening for susceptibility syndromes associated with WT and its proper managements in children.

Wilms tumorSyndromeScreeningTherapy

孙中文、王富强、徐青雨、王慧智、池成涛、马志

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牡丹江医学院,牡丹江 157011

河南宏力医院心胸外科,新乡 453499

牡丹江医学院附属红旗医院小儿外科,牡丹江 157011

牡丹江医学院附属红旗医院妇产科,牡丹江 157011

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肾母细胞瘤 综合征 筛查 治疗

2021年度黑龙江省卫生健康委科研课题

20210404130288

2024

中华小儿外科杂志
中华医学会

中华小儿外科杂志

CSTPCD北大核心
影响因子:0.853
ISSN:0253-3006
年,卷(期):2024.45(2)
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