ARAR2331: Prospective Treatment of Types I, II and III Pleuropulmonary Blastoma (PPB)
ARAR2331
What is the goal of the study?
PPB is a rare malignancy but the most common primary lung tumor in young children. It has not been previously prospectively studied within the COG or other large consortia. This trial employs a VTC window for Type II and III tumors, based on preclinical evidence of activity of camptothecins. Patients are then assigned, based on response, to receive treatment with one of two regimens employing the more standard IVADo chemotherapy. The primary aim is to determine the ORR to two cycles of VTC in patients with Type II or III disease. The secondary aims are to estimate 3 yr OR and EFS in Type I patients treated with surgery alone or surgery and chemotherapy following standardized guidelines and to estimate the 3 yr OR and EFS of patients with Type II and III PPB. Exploratory aims include evaluating the molecular features of PPB and correlating with outcome, biobanking tissue and serum, ctDNA, primary resection rate, and surgical complications.
Who can participate in the study?
Please contact the study team listed below to learn more.