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目的 探讨小儿胸膜肺母细胞瘤 (PPB)的诊断、治疗及预后。方法 回顾 2 2例小儿胸膜肺母细胞瘤病例 ,按Dehuer病理分型为II型者 15例、III型 7例 ,对其进行随访。结果 2 2例年龄 3个月～ 10岁 ,男女比例 1∶1。最早出现的症状均为咳嗽 ,发热 19例 ,呼吸困难 13例。早期X线表现 :肺囊性病 4例 ,气胸 1例 ,胸腔斑、片状影 11例 ,胸腔积液 4例 ,纵隔占位 2例。来院前首诊 (县、市级医院 )误诊率 10 0 % ,在我院首诊误诊率 77 3% ,首诊到确诊时间平均 (5 4± 6 2 )个月。出现症状到确诊时间0 5～ 2 4个月。手术 18例 ,术后均化疗。随访 1个月～ 12年。生存 9例 ,2年生存率 2 7 3% ,5年生存率9 1%。死亡 13例 ,诊断到死亡时间 1d～ 2 6个月。结论 小儿肺母细胞瘤无论在组织学、遗传学还是在临床表现及预后与成人肺母细胞瘤 (PB)有明显不同 ,是一种少见的极易误诊的儿童期恶性肿瘤。早期确诊、治疗是决定预后的关键。
Objective To investigate the diagnosis, treatment and prognosis of pediatric pleuropulmonary blastoma (PPB). Methods Twenty-two cases of pediatric pleuropneumocytoma were retrospectively reviewed. Fifteen cases of type II and 7 cases of type III were selected according to Dehuer’s pathology. The patients were followed up. RESULTS: Twenty-two patients, aged 3 months to 10 years old, had a male to female ratio of 1: 1. The first symptoms were cough, fever in 19 cases, 13 cases of dyspnea. Early X-ray findings: 4 cases of pneumocystic disease, pneumothorax in 1 case, pleural plaque, patchy film in 11 cases, pleural effusion in 4 cases, mediastinal mass in 2 cases. The first visit to the hospital (county, municipal hospitals) misdiagnosis rate of 10%, the first visit in our hospital misdiagnosis rate of 77 3%, the first diagnosis to an average of (54 ± 6 2) months. Symptoms to the time of diagnosis 0 5 ~ 2 4 months. 18 cases of surgery, postoperative chemotherapy. Followed up for 1 month ~ 12 years. 9 cases survived, 2-year survival rate was 273%, and 5-year survival rate was 9 1%. Thirteen died, diagnosed to death time 1d ~ 26 months. Conclusions Pediatric pneumoblastoma is a rare and extremely misdiagnosed childhood malignancy in both histology, genetics, clinical manifestations and prognosis, and is significantly different from adult pulmonary blastoma (PB). Early diagnosis and treatment is the key to determine the prognosis.