Children with type II and III pleuropulmonary blastoma (PPB) treated with ifosfamide, vincristine, actinomycin-D, and doxorubicin (IVADo) appeared to have similar outcomes as a historical control group, according to research published in the Journal of Clinical Oncology.

Researchers analyzed data on 314 patients from the International PPB/DICER1 Registry ( Identifier: NCT03382158) who had type II PPB (173 patients) or type III PPB (141 patients). The median age at baseline was 36.9 months (range, 3.8-235.6 months).

There were 132 patients who received IVADo from 2007 to 2021, and there were 182 patients in the historical control cohort who were treated from 1973 to 2021.

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When comparing IVADo with historical regimens, the adjusted hazard ratio (HR) was 0.77 (95% CI, 0.51-1.18) for event-free survival and 0.65 (95% CI, 0.39-1.08) for overall survival.

However, when the researchers adjusted for trends in time, the adjusted HR was 0.95 (95% CI, 0.57-1.59) for event-free survival and 0.98 (95% CI, 0.53-1.83) for overall survival.

The researchers noted that more than one quarter of children with type II PPB and nearly half of children with type III PPB will die as a result of their cancer.

“Novel strategies to prevent on-treatment and post-treatment recurrence are needed,” the researchers wrote, adding that they encourage future international collaboration to work toward better treatments for children with advanced PPB.

Disclosures: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of disclosures.


Schultz KAP, Harris AK, Nelson AT, et al. Outcomes for children with type II and type III pleuropulmonary blastoma following chemotherapy: A report from the International PPB/DICER1 Registry. J Clin Oncol. Published online September 22, 2022. doi:10.1200/JCO.21.02925

This article originally appeared on Oncology Nurse Advisor