Type I PPB: Treatment Issues

Priest JR, Hill DA, Williams GM, Moertel CM, Messinger Y, Finkelstein MJ, Dehner LP. Type I pleuropulmonary blastoma: A report from the International Pleuropulmonary Blastoma Registry. J Clin Oncol 2006;24:4492-8. The International PPB/DICER1 Registry has collected a series of Type I PPB cases and analyzed outcome based on treatments of surgery with or without adjuvant chemotherapy. Recurrences in the surgery alone group were 8/20 (40%) and in the adjuvant chemotherapy group were 1/17 (6%). Recurrence-free survival is statistically greater with adjuvant chemotherapy. Overall survival is not significantly different. All recurrences were Type II or III PPB. Survival in the adjuvant chemotherapy group was approximately 90%. Survival in the surgery alone group was about 65%. There may be some cases of Type I PPB that can be safely followed by close monitoring, and the Registry is studying Type I PPB to try to identify prognostic factors that can predict who benefits from adjuvant chemotherapy. To learn more, see also For Doctors-Recommendations-Treatment- Type I PPB. Clinicians are encouraged to contact the Registry for the latest information.