胸膜肺母细胞瘤(PPB)是儿童中非常罕见且高度侵袭性的肿瘤,大多在6岁以下。我们评估了临床特征,治疗方式,治疗结果,以及影响在我们机构接受治疗的PPB患者生存的预后因素,以改善预后。
从2008年11月到2019年11月,31名儿童(21名男孩和10名女孩),年龄中位数为30个月(范围,22天至54个月)在我们的机构接受治疗。这里我们描述病人的特征,治疗方式,和治疗结果。Kaplan-Meier方法用于评估无进展生存期(PFS)和总生存期(OS)。进行对数秩检验以进行组间比较。
3名儿童失访,2名因术后并发症死亡。在纳入随访的26例患者中,16例PPB患者显示无瘤生存期。5年PFS和OS分别为60.4%和60.1%。通过分层统计分析,I型PPB的5年PFS和OS为100%,而III型PPB分别为43.7%和43%,分别。肿瘤完全切除的5年PFS和OS分别为76.5%和75.6%,分别,而肿瘤残留者为31.3%。合并化疗的5年PFS和OS分别为62.2%和61.6%,分别,无化疗者为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,术后辅助治疗。
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.