Treatment and prognosis of pleuropulmonary blastoma: A Single-Center Report of 31 Cases

Objectives: Pleuropulmonary blastoma (PPB) is a very rare, characteristic and highly aggressive neoplasm occurring in children, most 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 of PPB. Methods: From November 2008 to November 2019, 31 children (21 boys and 10 girls) with a median age of 30 months (range, 22 days-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 probability (PFS) and overall survival (OS). Log-rank test was performed for comparison between groups. Results: 3 children were lost to follow-up and 2 were dead of postoperative complications. Of the 26 patients included in the follow-up, 16 PPB patients displayed tumor-free survival. The 6-month, 1-, 3-, and 5-year PFS were 80.8%, 69.0%, 60.4% and 60.4%, respectively. Accordingly, the 6-month, 1-, 3-, and 5-year OS were 84.6%, 72.7%, 60.1% and 60.1%, respectively. Sex, extent of surgery and chemotherapy/irradiation appeared to affect the survival, while age and pathology type appeared not to do. Conclusions: PPB is an aggressive neoplasm. To improve the prognosis of PPB, we should promote radical resection and improve the auxiliary treatment measures.