儿童纵隔畸胎瘤156例临床特征及外科治疗

Clinical manifestations and surgical treatment of pediatric mediastinal teratoma:Retrospective analysis of 156 cases

目的 总结儿童纵隔畸胎瘤的临床特征、诊断要点及外科治疗经验。方法 以2012年1月至2021年12月首都医科大学附属北京儿童医院胸外科收治的156例纵隔畸胎瘤患儿为研究对象,患儿均经病理学检查确诊为纵隔畸胎瘤。男74例,女82例;年龄17天至15岁9个月(平均年龄6岁3个月)。肿瘤位置:前纵隔129例,后纵隔22例(合并椎管内畸胎瘤10例),中纵隔(心包)2例,肺2例,食管1例。结果 临床表现:咳嗽发热79例,胸痛45例,胸闷气促40例,无症状10例,下肢无力或瘫痪4例,咯血2例,胸壁畸形2例,咳出异物1例,心脏杂音1例,腹痛1例。156例均行肿瘤切除术,其中机器人手术3例(1.9%),胸腔镜手术65例(41.7%),胸腔镜中转开胸手术16例(10.3%),开胸手术72例(46.2%)。142例为成熟畸胎瘤,14例为未成熟畸胎瘤(Ⅰ级7例,Ⅱ级5例,Ⅲ级2例)。术后发生心包积液1例,乳糜漏2例,膈膨升6例。患儿均治愈出院,术后平均随访60个月(1~121个月),1例术后复发,经再次手术切除。结论 儿童纵隔畸胎瘤临床表现、影像学特征较为复杂,确诊需结合临床表现及MRI、CT、超声等影像学检查结果。手术是儿童纵隔畸胎瘤安全有效的治疗方案,早期诊断并根据肿瘤临床特征选择合理的手术时机和治疗方式是获得良好预后的关键。

Objective To summarize the clinical manifestations and surgical strategies of pediatric mediastinal teratoma(MT).Methods 156 cases of MT diagnosed with surgery and pathological results from January,2012 to December,2021 were recruited.There were 74 males and 82 females with the mean age of 6.25 years old(17 days-15.75 years).The patients’ tumors located in the anterior mediastinum(n=129),posterior mediastinum(n=22,including 10 in vertebral canal),pericardium(n=2),lung(n=2),and esophagus(n=1).Results Clinical manifestations of the total 156 patients:cough(n=79),chest pain(n=45),chest tightness(n=40),no symptoms(n=10),lower limbs myasthenia(n=4),hemoptysis(n=2),chest wall deformity(n=2),coughing up foreign body(n=1),cardiac murmur(n=1),and abdominal pain(n=1).The procedures of tumor resection were performed via robotic surgery(n=3,1.9%),thoracoscopic surgery(n=65,41.7%),thoracoscopic surgery transfered to thoracotomy(n=16,10.3%),and thoracotomy(n=72,46.2%).All cases were confirmed with MT by postoperative pathological examination,of which 142 were mature teratoma,14 were immature teratoma(including 7 cases of grade WHO Ⅰ,2 cases of grade WHO Ⅱ,and 2 cases of grade WHO Ⅲ).The average follow-up time was 60 months(1-121 months),one patient suffered postoperative recurrence and received the second operation.There were 1 case of pericardial effusion,2 cases of chylothorax,and 6 cases of diaphragmatic eventration after surgery.Conclusion Pediatric MT has complex clinical symptoms and imaging features,which requires to combine the clinical manifestations with imaging findings of MRI,CT,ultrasound for accurate diagnosis.Complete excision of mature teratoma has a favorable prognosis.In summary,surgical treatment is a safe and effective method for children with MT.Early diagnosis and ideal surgery timing and treatment based on the clinical feature of the teratoma are the key to obtain good prognosis.