Department of Thoracic Surgery, Guangzhou Women and Children's Medical Centre, Guangzhou, Guangdong, China.
Pediatr Pulmonol. 2022 Sep;57(9):2237-2243. doi: 10.1002/ppul.25930. Epub 2022 Jun 13.
Pleuropulmonary blastoma (PPB) is a very rare and highly aggressive neoplasm occurring in children, mostly under 6 years of age. We assessed the clinical characteristics, treatment modalities, treatment outcomes, and prognostic factors affecting survival in patients with PPB treated at our institution over a 10-year period to improve the prognosis.
From November 2008 to November 2019, 31 children (21 boys and 10 girls) with a median age of 30 months (ranging, 22 days to 54 months) were treated at our institution. Here we describe the patient characteristics, treatment modalities, and treatment outcomes. The Kaplan-Meier method was used to estimate the progression-free survival (PFS) and overall survival (OS). Log-rank test was performed for comparison between groups.
Three children were lost to follow-up and two were dead due to postoperative complications. Of the 26 patients included in the follow-up, 16 PPB patients displayed tumor-free survival. The 5-year PFS and OS were 60.4% and 60.1% respectively. By stratified statistical analysis, the 5-year PFS and OS of type I PPB were 100%, while those of type III PPB were 43.7% and 43%, respectively. The 5-year PFS and OS of complete tumor resection were 76.5% and 75.6%, respectively, while those with tumor residue were 31.3%. The 5-year PFS and OS combined with chemotherapy were 62.2% and 61.6%, respectively, while those without chemotherapy were 0%.
PPB is an aggressive neoplasm. The main factors related to the prognosis of PPB are pathological type, tumor resection degree, and postoperative adjuvant therapy.
胸膜肺胚细胞瘤（PPB）是一种非常罕见且高度侵袭性的肿瘤，主要发生在 6 岁以下的儿童中。我们评估了在我们机构治疗的 10 年期间患有 PPB 的患者的临床特征、治疗方式、治疗结果以及影响生存的预后因素，以改善预后。
2008 年 11 月至 2019 年 11 月，在我们机构治疗了 31 名儿童（21 名男孩和 10 名女孩），中位年龄为 30 个月（范围为 22 天至 54 个月）。在这里，我们描述了患者的特征、治疗方式和治疗结果。使用 Kaplan-Meier 方法估计无进展生存期（PFS）和总生存期（OS）。对数秩检验用于组间比较。
3 名儿童失访，2 名因术后并发症死亡。在纳入随访的 26 名患者中，16 名 PPB 患者显示肿瘤无复发生存。5 年 PFS 和 OS 分别为 60.4%和 60.1%。分层统计分析显示，I 型 PPB 的 5 年 PFS 和 OS 为 100%，而 III 型 PPB 分别为 43.7%和 43%。完全肿瘤切除的 5 年 PFS 和 OS 分别为 76.5%和 75.6%，而肿瘤残留者分别为 31.3%。联合化疗的 5 年 PFS 和 OS 分别为 62.2%和 61.6%，而未化疗者为 0%。
PPB 是一种侵袭性肿瘤。与 PPB 预后相关的主要因素是病理类型、肿瘤切除程度和术后辅助治疗。
