Type I and Ir pleuropulmonary blastoma (PPB): A report from the International PPB/DICER1 Registry.
Alexander T NelsonAnne K HarrisDave WatsonDoug MiniatiMike FinchJunne KamiharaSarah G MitchellDavid B WilsonKatie GettingerArun A RangaswamiJosé M CamposSara LedermanBrad A FeltisLauren M VastaLaura A HarneyDouglas R StewartLouis P DehnerYoav H MessingerD Ashley HillKris Ann P SchultzPublished in: Cancer (2022)
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.