Skip to main content
Download PDF
- Main
Type I and Ir pleuropulmonary blastoma (PPB): A report from the International PPB/DICER1 Registry
- Nelson, Alexander T;
- Harris, Anne K;
- Watson, Dave;
- Miniati, Doug;
- Finch, Mike;
- Kamihara, Junne;
- Mitchell, Sarah G;
- Wilson, David B;
- Gettinger, Katie;
- Rangaswami, Arun A;
- Campos, José M;
- Lederman, Sara;
- Feltis, Brad A;
- Vasta, Lauren M;
- Harney, Laura A;
- Stewart, Douglas R;
- Dehner, Louis P;
- Messinger, Yoav H;
- Hill, D Ashley;
- Schultz, Kris Ann P
- et al.
Published Web Location
https://doi.org/10.1002/cncr.34593Abstract
Background
Pleuropulmonary blastoma (PPB) is the most common lung cancer of infancy and early childhood. Type I PPB is a purely cystic lesion that has a microscopic population of primitive small cells with or without rhabdomyoblastic features and may progress to type II or III PPB, whereas type Ir lacks primitive small cells.Methods
Children with suspected PPB were enrolled in the International PPB/DICER1 Registry. Pathology was centrally reviewed, and follow-up was ascertained annually.Results
Between 2006 and 2022, 205 children had centrally reviewed type I or Ir PPB; 39% of children with type I and 5% of children with type Ir PPB received chemotherapy. Outcomes were favorable, although 11 children (nine with type I and two with type Ir PPB) experienced progression to type II/III (n = 8) or regrowth of type I PPB at the surgical site (n = 3), none of whom received chemotherapy before progression. Age and cyst size in combination were more suitable than either factor alone in predicting whether a particular lesion was type I or Ir PPB.Conclusions
For young children with type I PPB, outcomes are favorable, but complete resection is indicated because of the risk for progression. Chemotherapy may be useful in a subset of children at increased risk for recurrence/progression. Efforts to risk stratify children with type I PPB to optimize outcomes while reducing treatment-related side effects are underway.Many UC-authored scholarly publications are freely available on this site because of the UC's open access policies. Let us know how this access is important for you.
Main Content
For improved accessibility of PDF content, download the file to your device.
Enter the password to open this PDF file:
File name:
-
File size:
-
Title:
-
Author:
-
Subject:
-
Keywords:
-
Creation Date:
-
Modification Date:
-
Creator:
-
PDF Producer:
-
PDF Version:
-
Page Count:
-
Page Size:
-
Fast Web View:
-
Preparing document for printing…
0%