High-Risk Children and Children with PPB

The following surveillance recommendations are based on patterns of metastasis and timing of recurrences of PPB: PPB tends to occur in children with lung cysts during their 3rd and 4th years of life. Recurrences of Types II and III PPB tend to occur within 24 months of diagnosis, and, with a few exceptions, almost all recurrences have occurred within 36 months of diagnosis.


Surveillance of Family Members

The PPB/DICER1 Registry does not make any recommendation about surveillance of siblings of a child with PPB. The difficulty is exemplified by the case reported by Delahunt [Delahunt B. et al. Familial cystic nephroma and pleuropulmonary blastoma. Cancer 1993;71(4):1338-42.]: a child whose two siblings had cystic nephroma had a surveillance CXR which was normal; 10 months later the child had a massive chest mass which was PPB. In one family we have followed, surveillance chest xrays were done on apparently-normal siblings, but this practice raises obvious questions about the frequency of surveillance and about causing anxiety instead of providing reassurance.