儿童恶性肿瘤并上腔静脉综合征50例临床分析

Clinical analysis of 50 children with superior vena cava syndrome combined with malignant tumors

目的总结分析儿童恶性肿瘤并上腔静脉综合征(SVCS)的临床特点、治疗及预后,提高临床医师对此病的认识。方法回顾性分析郑州大学第一附属医院2010年11月至2022年5月收治的50例恶性肿瘤并SVCS患儿的临床资料,总结病理类型、临床表现、影像学检查、治疗及预后。采用Kaplan-Meier法评估患儿总生存率和无事件生存率。结果50例患儿中,男38例,女12例,男女比例3.2∶1.0,发病中位年龄12.5(8.0,14.5)岁,以青春期起病最常见(66.0%,33/50)。咳嗽(80.0%,40/50)是最常见的临床表现,其余依次是面颈部水肿(66.0%,33/50)、胸闷(56.0%,28/50)、呼吸困难(50.0%,25/50)。50例患儿均经组织病理学检查确诊,非霍奇金淋巴瘤(NHL)39例(78.0%),是最常见的恶性肿瘤,其中T淋巴母细胞淋巴瘤(T-LBL)占74.4%(29/39)。50例患儿均行CT检查,纵隔占位42例(84.0%),合并胸腔积液(86.0%,43/50)及心包积液(70.0%,35/50)常见。50例患儿3年累积生存率和无事件生存率分别为59.7%和57.9%,39例NHL患儿3年累积生存率和无事件生存率分别为62.9%和60.9%。结论儿童恶性肿瘤相关SVCS起病急,病情进展快,预后差。病因以NHL最常见,尤其是T-LBL,咳嗽、面颈部水肿、胸闷、呼吸困难是常见的临床表现。早期发现,及早治疗可能挽救患儿生命。

Objective To summarize and analyze the clinical characteristics,treatment and prognosis of superior vena cava syndrome(SVCS)with malignant tumors in children,and to improve the understanding of its clinical management.Methods Clinical data of 50 children with SVCS combined with malignant tumors treated in the First Affiliated Hospital of Zhengzhou University from November 2010 to May 2022 were analyzed retrospectively.The pathological types,clinical manifestations,imaging examination,treatment and prognosis were summarized.The overall survival(OS)rate and event-free survival(EFS)rate were evaluated by Kaplan-Meier method.Results Among the 50 cases,38 were males and 12 were females,with a male/female ratio of 3.2∶1.0.The median onset was 12.5(8.0,14.5)years,and the most common onset occurred in adolescence(66.0%,33/50).Cough(80.0%,40/50)was the most common clinical manifestation,followed by face and neck edema(66.0%,33/50),chest tightness(56.0%,28/50)and dyspnea(50.0%,25/50).All the 50 cases were confirmed by histopathological examination,39 cases(78.0%)were diagnosed as non-Hodgkin′s lymphoma(NHL).NHL was the most common malignant tumor,of which T-lymphoblastic lymphoma(T-LBL)accounted for 74.4%(29/39).All the 50 cases were examined by CT examination,involving 42 cases(84.0%)detected with mediastinal masses.Pleural effusion(86.0%,43/50)and pericardial effusion(70.0%,35/50)were common imaging findings.The 3-year OS rate and EFS rate of them were 59.7%and 57.9%,respectively.The 3-year OS rate and EFS rate of the 39 children with NHL were 62.9%and 60.9%,respectively.Conclusions Children with malignant tumors complicated with SVCS are featured by the acute onset,rapid progress and poor prognosis.NHL is the most common cause,especially T-LBL.Cough,edema of face and neck,chest tightness and dyspnea are common clinical manifestations.Early detection and treatment contribute to save children′s lives.