儿童纵隔恶性肿瘤相关上腔静脉综合征42例分析

Analysis of 42 cases of childhood superior vena cava syndrome associated with mediastinal malignancy

目的总结儿童纵隔恶性肿瘤相关上腔静脉综合征(SVCS)的临床特点、处理方法及预后。方法回顾性总结2015年1月至2021年12月上海儿童医学中心收治的42例纵隔恶性肿瘤相关SVCS患儿的临床资料, 对其临床表现、原发病类型、原发病诊断过程以及预后情况进行总结。结果 42例纵隔恶性肿瘤相关SVCS患儿中男31例、女11例, 诊断年龄8.5(1.9, 14.9)岁。临床表现以咳喘(33例, 79%)、端坐呼吸(19例, 45%)、面部水肿(18例, 43%)较为常见。病理诊断以T细胞性淋巴母细胞淋巴瘤(T-LBL)最常见(25例, 60%), 其他依次为T细胞急性淋巴细胞白血病(T-ALL)(7例, 17%)、间变性大细胞淋巴瘤(4例, 10%)、弥漫大B细胞淋巴瘤2例(5%), 外周T淋巴瘤、霍奇金淋巴瘤、尤文肉瘤、生殖细胞瘤各1例。42例患儿通过骨髓细胞学或胸腔积液细胞学明确原发病诊断14例, 通过浅表淋巴结活检确诊6例, 通过纵隔占位性病灶活检确诊22例;麻醉方式以局部麻醉为主(27例, 64%), 15例接受全身麻醉患儿中3例发生纵隔肿块压迫导致的呼吸相关并发症;42例患儿中27例持续缓解, 1例复发后接受二线治疗存活, 14例死亡(2例死于活检术围手术期并发症, 12例原发病复发或进展后死亡), 持续缓解的27例患儿随访时间36.7(1.2, 76.1)个月。42例患儿3年总生存率和无事件生存率分别为59%(95%CI 44%~79%)和58%(95%CI 44%~77%)。结论儿童纵隔恶性肿瘤相关SVCS是危及生命的肿瘤急症, 病死率高。最常见原发疾病是T-LBL, 临床症状及体征以咳喘、端坐呼吸、面部水肿常见。临床处理应以稳定危重状态为前提并通过最小侵袭性操作以明确病理诊断。

Objective To summarize the clinical features,management and outcome of superior vena cava syndrome(SVCS)associated with mediastinal malignancy in children.Methods Clinical data of 42 children of SVSC associated with mediastinal malignancy in Shanghai Children′s Medical Center from January 2015 to December 2021 were collected and analyzed retrospectively.The clinical manifestations,pathological diagnosis,disease diagnosis process,and prognosis were summarized.Results Among 42 children of SVCS associated with mediastinal malignancy,there were 31 males and 11 females.The age at diagnosis was 8.5(1.9,14.9)years.Cough and wheezing(33 cases,79%),orthopnea(19 cases,45%)and facial edema(18 cases,43%)occurred most commonly.T-cell lymphoblastic lymphoma(T-LBL)was the most frequent pathological diagnosis(25 cases,60%),followed by T-cell acute lymphoblastic leukemia(T-ALL)(7 cases,17%),anaplastic large cell lymphoma(4 cases,10%)and diffuse large B-cell lymphoma(2 cases,5%),peripheral T-lymphoma,Hodgkin lymphoma,Ewing′s sarcoma and germ cell tumor(1 case each).Pathological diagnosis was confirmed by bone marrow aspiration or thoracentesis in 14 cases,peripheral lymph node biopsy in 6 cases,and mediastinal biopsy in 22 cases.Twenty-seven cases(64%)had local anesthesia.Respiratory complications due to mediastinal mass developed in 3 of 15 cases who received general anesthesia.Of the 42 cases,27 cases had sustained remission,1 case survived with second-line therapy after recurrence,and 14 cases died(2 cases died of perioperative complications and 12 cases died of recurrence or progression of primary disease).The follow-up time was 36.7(1.2,76.1)months for 27 cases in continuous complete remission.The 3-year overall survival(OS)and events free survival(EFS)rates of 42 children were 59%(95%CI 44%-79%)and 58%(95%CI 44%-77%)respectively.Conclusions SVCS associated with mediastinal malignancy in children is a life-threatening tumor emergency with high mortality.The most common primary disease is T-LBL.The most common clinical symptoms and signs are cough,wheezing,orthopnea and facial edema.Clinical management should be based on the premise of stable critical condition and confirm the pathological diagnosis through minimal invasive operation.