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  • This data is not published and is not peer-reviewed.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
Overall Survival TypesI, II, and III

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
Recurrance Free TypeI, II, and III

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.
Child Overall Survival Type III

months for PPB diagnosis

Overall Survival: Type II PPB

After 1995 green, before 1995 blue; p value for difference: 0.151
Child Overall Survival Type II

months from PPB diagnosis