28例小儿胸膜肺母细胞瘤的临床病理特征及预后

Clinicopathologic features and prognosis of 28 cases of pleuralpulmonary blastoma in children

目的:探讨小儿胸膜肺母细胞瘤的临床病理特征、治疗方法和预后。方法:回顾性分析2008年11月至2018年5月广州市妇女儿童医疗中心胸外科收治的28例胸膜肺母细胞瘤患者的临床病理特征。28例患者中,男18例,女10例;年龄22 d至5岁10个月,平均年龄2岁6个月。结果:28例患者中,行活检术1例;手术治疗27例,其中14例Ⅱ、Ⅲ型胸膜肺母细胞瘤术后接受化疗。Ⅰ型胸膜肺母细胞瘤5例,Ⅱ型胸膜肺母细胞瘤5例,Ⅲ型胸膜肺母细胞瘤18例。24例患者获得有效随访,其中,无瘤生存15例;3例接受化疗患儿分别在术后6、10、18个月复发;1例接受化疗患儿在术后11个月出现髂骨转移;2例未接受化疗患儿分别在术后2个月出现肿瘤复发、3个月出现右胫骨及左肾转移;3例自动出院患儿均在出院后1个月内死亡。结论:胸膜肺母细胞瘤恶性程度高,进展快,Ⅰ型胸膜肺母细胞瘤预后较好,Ⅱ、Ⅲ型胸膜肺母细胞瘤预后较差,术后化疗可能提高患儿的生存率。

Objective To explore the clinical characteristics,therapeutic methods and prognosis of pleuropulmonary blastoma in children.Methods The clinical data of 28 patients with pleuropulmonary blastoma diagnosed in Guangzhou Women and Children′s Medical Centre from November 2008 to May 2018 were collected and retrospectively analyzed.Results Of the 28 patients,18 were male and 10 were female,aged from 22 days to 5 years 10 month,the average age was 2 years 6 months.One patient underwent biopsy and other 27 underwent operation,14 patients with type Ⅱ/Ⅲ pleuropulmonary blastoma received postoperative chemotherapy.Five patients were pathologically diagnosed as type Ⅰ pleuropulmonary blastoma,5 were type Ⅱ pleuropulmonary blastoma and 18 were type Ⅲ pleuropulmonary blastoma.During the follow-up period of 24 patients,15 patients were disease free survival,3 patients relapsed within 6 months,10 months and 18 months after chemotherapy,respectively.One patient who received postoperative chemotherapy suffered a bone metastasis within 11 months,2 patient without chemotherapy relapsed within 2 months and suffered bone or renal metastasis within 3 months,respectively.Three patients who left hospital voluntarily died within 1 month.Conclusions Pleuropulmonary blastoma is a highly malignant and rapidly progressed neoplasm.Patients with type Ⅰ pleuropulmonary blastoma have good prognoses while the prognoses of Ⅱ/Ⅲ pleuropulmonary blastoma are poor.Postoperative chemotherapy seems to improve the survival of patients with Ⅱ/Ⅲ pleuropulmonary blastoma.