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The International Pleuropulmonary Blastoma Registry
The International Pleuropulmonary Blastoma Registry
The Genetics of PPB

Critical Issues


Constitutional & Familial Issues: Genetics of PPB:


A Very Rare Disease: Enrolling Patients in the Registry


Cerebral Metastases in PPB


Genetic Study of PPB


PPB Associated with Cystic Nephroma and Other Renal Tumors


Type I PPB: Treatment Issues


Type II and III THERAPY RECOMMENDATION CHANGE

Join Our PPB Study

Whats New

04/09/2010
2010 PPB FAMILY WEEKEND IN WASHINGTON DC

02/15/2007
THERAPY CHANGE: PPB Types II and III

11/15/2006
PPB, Cystic Nephroma & Small Bowel Polyps

10/01/2005
Genetic Study in PPB - PPB Tissue Bank

Recommendations
Surveillance Recommendations – 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 Recommendations Diagram
Surveillance of Family Members

The PPB 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 [see Bibliography]: 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.

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Data and recommendations on this site are conscientiously presented but some are unpublished observations and have not undergone peer review.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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