loading page

Treatment and prognosis of pleuropulmonary blastoma: A Single-Center Report of 31 Cases
  • +5
  • Feng Wang,
  • Lin Zhuang,
  • Jianghua Liang,
  • Le Li,
  • Hui Wang,
  • Jue Tang,
  • Jia Zeng,
  • Wei Liu
Feng Wang
Guangzhou Women and Children’s Medical Center

Corresponding Author:[email protected]

Author Profile
Lin Zhuang
Guangzhou Women and Children's Medical Center
Author Profile
Jianghua Liang
Guangzhou Women and Children’s Medical Center
Author Profile
Le Li
Guangzhou Women and Children’s Medical Center
Author Profile
Hui Wang
Guangzhou Women and Children's Medical Center
Author Profile
Jue Tang
Guangzhou Women and Children’s Medical Center
Author Profile
Jia Zeng
Guangzhou Women and Children’s Medical Center
Author Profile
Wei Liu
Guangzhou Women and Children’s Medical Center
Author Profile

Abstract

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.
12 Mar 2021Submitted to Pediatric Pulmonology
15 Mar 2021Submission Checks Completed
15 Mar 2021Assigned to Editor
04 Sep 2021Reviewer(s) Assigned
29 Oct 2021Review(s) Completed, Editorial Evaluation Pending
31 Oct 2021Editorial Decision: Revise Major
10 Dec 20211st Revision Received
25 Jan 2022Reviewer(s) Assigned
25 Jan 2022Submission Checks Completed
25 Jan 2022Assigned to Editor
07 Mar 2022Review(s) Completed, Editorial Evaluation Pending
08 Mar 2022Editorial Decision: Revise Minor
24 Mar 20222nd Revision Received
25 Mar 2022Submission Checks Completed
25 Mar 2022Assigned to Editor
25 Mar 2022Reviewer(s) Assigned
04 Apr 2022Review(s) Completed, Editorial Evaluation Pending
04 Apr 2022Editorial Decision: Revise Minor
08 Apr 20223rd Revision Received
09 Apr 2022Submission Checks Completed
09 Apr 2022Assigned to Editor
10 Apr 2022Review(s) Completed, Editorial Evaluation Pending
10 Apr 2022Editorial Decision: Accept
13 Jun 2022Published in Pediatric Pulmonology. 10.1002/ppul.25930