Indolfi Paolo, Bisogno Gianni, Casale Fiorina, Cecchetto Giovanni, De Salvo Gianluca, Ferrari Andrea, Donfrancesco Alberto, Donofrio Vittoria, Martone Antonio, Di Martino Martina, Di Tullio Maria T
Pediatric Oncology Service Second University of Naples, Italy.
Pediatr Blood Cancer. 2007 Mar;48(3):318-23. doi: 10.1002/pbc.20842.
To evaluate the prognostic factors in a series of children affected by pleuropulmonary blastoma (PPB).
Clinicopathological findings, treatment, and outcome of 22 PPB cases observed in 13 Italian Associations for Pediatric Hematology and Oncology centers are reported. Clinical data, surgical notes, pathologic findings, and summaries of treatment were taken from the charts and correlated with outcome by standard statistical methods.
The series included 22 patients (14 males) with a median age of 30.5 months followed up for a median of 22 months (range 2-176 months). In nine patients the PPB developed with lung involvement only. Congenital lung cysts were recorded in five cases. Nine patients had recurrences. Gender, side, tumor size, pre-existing lung cysts, and extent of surgical resection at diagnosis did not significantly affect survival by univariate analysis. Achieving total resection of the tumor at any time of treatment resulted in a significantly better prognosis (P = 0.01), whereas extrapulmonary involvement at diagnosis resulted in a significantly worse prognosis (P = 0.01). Estimated 15-year event-free and overall survival rates were 44 and 49% for all patients, respectively.
PPB is an aggressive neoplasm. Total resection of PPB performed at any time of treatment appears to provide a better outcome, whereas extrapulmonary involvement at diagnosis worsens the prognosis.
评估一系列胸膜肺母细胞瘤（PPB）患儿的预后因素。
报告了在13个意大利儿科血液学和肿瘤学协会中心观察到的22例PPB病例的临床病理特征、治疗情况及预后。临床资料、手术记录、病理结果和治疗总结均取自病历，并通过标准统计学方法与预后进行关联分析。
该系列包括22例患者（14例男性），中位年龄30.5个月，中位随访时间22个月（范围2 - 176个月）。9例患者的PPB仅累及肺部。5例记录有先天性肺囊肿。9例患者出现复发。单因素分析显示，性别、患侧、肿瘤大小、术前存在的肺囊肿以及诊断时手术切除范围对生存率无显著影响。在治疗的任何阶段实现肿瘤全切可使预后显著改善（P = 0.01），而诊断时存在肺外受累则预后显著较差（P = 0.01）。所有患者的15年无事件生存率和总生存率分别估计为44%和49%。
PPB是一种侵袭性肿瘤。在治疗的任何阶段对PPB进行全切似乎可带来更好的预后，而诊断时存在肺外受累则会使预后恶化。
