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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

Critical Issues
Cerebral Metastases in PPB - Surveillance Recommended:

Cerebral parenchymal metastasis is frequent in PPB. IT OCCURS EVEN WHEN CHEST DISEASE IS UBDER CONTROL. (See Priest et al. Cerebral Metastasis and other central nervous system complications of pleuropulmonary blastoma. Pediatr Blood Cancer 2007; 49:266-273). Cerebral parenchymal metastasis is the most common form of hematogenous spread. It is more common in PPB than in any other childhood solid tumor. It occurs in Type II and Type III PPB. Life-table estimates of the probability of cerebral metastasis by 5 years from diagnosis are 54% in Type III PPB and 11% in Type II PPB. Cerebral metastases occur generally in the first two years after diagnosis but up to 5 years later. In 50% of cases of cerebral metastasis, the chest disease is under complete control. Cerebral metastasis has been successfully treated. The International PPB Registry recommends surveillance to discover cerebral metastases as early as possible. See For Doctors - Basics of PPB - Metastasis and For Doctors - Recommendations - Surveillance. Or contact the Registry for more information.
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Data and recommendations on this site are conscientiously presented but some are unpublished observations and have not undergone peer review.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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