Type I and Ir pleuropulmonary blastoma (PPB): A report from the International PPB/DICER1 Registry.

ABSTRACT:

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 progressio

SUBMITTER: Nelson AT 

PROVIDER: S-EPMC10316522 | biostudies-literature | 2023 Feb

REPOSITORIES: biostudies-literature