儿童肺部原发性肿瘤56例临床分析

Clinical analysis of primary lung tumors in 56 children

目的探讨儿童肺部原发性肿瘤的临床特征、治疗方案及预后。方法 2009—2019年间广州市妇女儿童医疗中心共收治儿童肺部原发性肿瘤患儿56例, 收集其一般资料、影像学资料、病理资料及手术记录等, 进行回顾性分析。结果 56例患儿中, 胸膜肺母细胞瘤28例, 炎性肌纤维母细胞瘤20例, 黏液表皮样癌6例, 血管瘤1例, 硬化性肺细胞瘤1例。临床表现以呼吸道症状为主, 咳嗽26例, 气促17例, 咳血3例。另外, 发热15例, 胸痛3例, 上腹部疼痛2例。肿瘤位于左肺下叶18例, 右肺下叶11例, 左肺上叶10例, 右肺上叶7例, 右肺中叶6例, 肺门4例。56例患儿均行手术治疗, 其中行开胸手术41例, 胸腔镜手术13例, 纤维支气管镜下手术2例。5例Ⅰ型胸膜肺母细胞瘤患儿术后未行化疗, 至随访结束仍存活。5例Ⅱ型胸膜肺母细胞瘤患儿中, 2例术后未化疗, 复发后死亡;3例术后化疗, 至随访结束仍存活。18例Ⅲ型胸膜肺母细胞瘤患儿术后均予IVADo方案化疗, 出现肿瘤复发6例, 远处转移3例, 肿瘤相关死亡8例。20例炎性肌纤维母细胞瘤患儿中, 行楔形切除术13例, 复发5例;行肺叶切除术6例, 复发1例;行纤维支气管镜手术1例, 术后复发。6例黏液表皮样癌患儿中, 行肺叶切除术5例, 行肺楔形切除术1例, 至随访结束均存活。1例血管瘤患儿行支气管镜下肿物切除术, 1例硬化性肺细胞瘤患儿行肺楔形切除术, 至随访结束均存活。结论儿童肺部原发性肿瘤缺乏特异性临床表现, 手术完整切除有利于取得良好预后。对于Ⅱ、Ⅲ型胸膜肺母细胞瘤, 化疗是必要的辅助治疗。

Objective To investigate the clinical manifestation,pathological type,treatment and prognosis of primary lung tumors in children.Methods We collected and retrospectively analyzed the clinical manifestation,pathological type,therapeutic method and prognosis of 56 primary lung tumors patients who diagnosed from 2009 to 2019 in Guangzhou Women and Children Medical Center.Results There were 56 patients identified as the primary lung tumors,including pleuropulmonary blastoma(PPB,n=28),pulmonary inflammatory myofibroblastic tumor(IMT,n=20),mucoepidermoid carcinoma(n=6),infantile hemangioma(n=1),pulmonary sclerosing hermangioma(n=1).Respiratory symptoms were the most manifestation at the time of diagnosis including 26 patients with cough,3 with hemoptysis,and 17 with dyspnea.Others included 15 with fever,3 with chest pain,and 2 with epigastiric pain.The primary tumor of 18 cases were located in the lower lobe of left lung,11 cases in the lower lobe of right lung,10 cases in the upper lobe of left lung,7 cases in the upper lobe of right lung,6 cases in the middle lobe of right lung,and 4 cases in pulmonary hilum.Among the 56 patients,41 patients underwent thoracotomy,13 thoracoscopy,and 2 fiberoptic bronchoscopy.Five patients with typeⅠPPB were still alive at the end of follow-up without chemotherapy.Among 5 patients with typeⅡPPB,2 patients without chemotherapy died after recurrence,3 patients suffered postoperative chemotherapy were still alive at the end of follow-up.All of the 18 patients with typeⅢPPB underwent postoperative chemotherapy with IVADo regimen.Recurrence occurred in 6 cases,distant metastasis occurred in 3 cases,and cancer-related deaths occurred in 8 cases.For 20 patients with IMT,recurrence occurred in 5 of 13 patients experienced wedge resection,1 of 6 patients experienced lobectomy and 1 of 6 underwent fiberoptic bronchoscopy,respectively.For 6 mucoepidermoid carcinoma patients,lobectomy was carried on 5 patients,wedge resection on 1 patient,all of them were still alive at the end of follow-up.One hermangioma patient underwent fiberoptic bronchoscopy and other 1 sclerosing hermangioma patient underwent wedge resection,both of them were still alive at the end of follow-up.Conclusions The clinical manifestations of the primary lung tumors in children are nonspecific.Complete resection and achieving negative marginattribute to the excellent outcome.Adjunctive treatment such as chemotherapy is necessary for patients with typeⅡand typeⅢPPB.