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The International Pleuropulmonary Blastoma Registry
The International Pleuropulmonary Blastoma Registry
Critical Issues
10/01/2005 - Genetic Study in PPB - PPB Tissue Bank:

The molecular basis of PPB is not known, but it appears to have a strong genetic component. PPB has early onset of disease, occurring primarily in infants and children less than five years of age. It is not unusual for children with PPB to have synchronous or metachronous tumors in the lung or other organs. First- and second-degree relatives of PPB patients also appear to be at increased risk for other pediatric embryonal neoplasms including rhabdomyosarcoma and Wilms tumor and related kidney tumors.(Delahunt et al., 1993; Priest et al., 1996). The goal of this research is to identify the genetic basis of PPB. Identifying the genetic basis of PPB will form the foundation of important future studies that will directly affect the care of children with PPB and also improve our understanding of the biology and pathogenesis of PPB and related childhood tumors. To learn more about the PPB Registry Genetic Study contact the Registry and go to the PPB Genetic Study website www.ppbgeneticstudy.org. We encourage all children with PPB and their families consider participating in this important research effort.
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Data and recommendations on this site are conscientiously presented but some are unpublished observations and have not undergone peer review. Consultation with the Registry is encouraged to clarify any topics. The International PPB Registry advises caution in the citation of website information.
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