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 (ClinicalTrials.gov 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.
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