Pleuropulmonary Blastoma: A Report on 350 Central Pathology–Confirmed Pleuropulmonary Blastoma Cases by the International Pleuropulmonary Blastoma Registry

Background Pleuropulmonary blastoma (PPB) has 3 subtypes on a tumor progression pathway ranging from type I (cystic) to type II (cystic/solid) and type III (completely solid). A germline mutation in DICER1 is the genetic cause in the majority of PPB cases.