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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

PPB Basic Facts
Prognosis:

As of mid-2009, International PPB Registry data for outcome in PPB is presented here. The data has the following features:

  • All cases are International PPB Registry cases with centrally-reviewed pathology.
  • The numbers of cases are as follows: Type I PPB: n = 68; Type II PPB n = 89, Type III PPB n = 82. [Type Ir (involuted/regressed Type I, n = 14) and Type II/III NOS (solid cases for which there is no definitive evidence for or against a cystic component, n = 4) cases are not included.]
  • The cases have been collected over 20+ years from many institutions and are heterogeneously treated.
  • Follow-up ranges from 0-477 months, median 26 months, mean 58 months.
  • Literature cases of PPB are included if they are also Registry pathology reviewed.
  • This data is not published and is not peer-reviewed.

OVERALL SURVIVAL Type I blue, Type II green, Type III grey

Statistics: Type I vs II vs III: p < 0.001; Type II vs III: p = 0.029


_____________________________________months from PPB diagnosis

RECURRENCE-FREE SURVIVAL Type I blue, Type II green, Type III grey

Statistics: Type I vs II vs III: p < 0.001; Type II vs III: p = 0.002

______________________________________months from ppb diagnosis


Survival for Children Treated BEFORE or AFTER 1995:

We arbitrarily analyzed treatment "success" for Types II and III PPB cases diagnosed in the last 15 years vs. earlier diagnosis. The results suggest that some progress is being made in effectiveness of treatment. These results further suggest that more recently treated cases are the best 'historical control' for evaluating the effectiveness of chemotherapy regimens being tried for PPB::

OVERALL SURVIVAL TYPE III PPB: after 1995 green, before 1995 blue; p value for difference: 0.068.

_____________________________________months for PPB diagnosis

OVERALL SURVIVAL TYPE II PPB: after 1995 green, before 1995 blue; p value for difference: 0.151

_____________________________________-months from PPB diagnosis


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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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