CA209-538 is a prospective multicentre Phase II clinical trial sponsored by the Olivia Newton-John Cancer Research Institute investigating the efficacy of combined immune checkpoint blockade (CTLA-4/ipilimumab and PD-1 blockade/nivolumab) in a range of rare cancers (NCT02923934, n=120). Participants received the anti-PD-1 antibody nivolumab and the anti-CTLA-4 antibody ipilimumab every three weeks for four doses, followed by nivolumab (3 mg/kg IV) every two weeks and continued for up to 96 weeks, until disease progression or unacceptable toxicity. Response was assessed every 12 weeks by RECIST version 1.1. Primary endpoint was clinical benefit rate (complete response, partial response, stable disease at 12 weeks). While approximately 20% of the study population progressed on or before the first evaluation at 12 weeks, the entire trial population achieved an overall response rate (ORR) and the clinical benefit rate (CBR) of ~ 30% and 50% respectively.
Patients with rare cancers have limited therapeutic options, low survival and are often excluded from clinical trials and biomarker studies due to the low incidence (<6 per 100,000 individuals). However, while incidence is low for each individual rare cancer, collectively they comprise of almost 25% of all cancer diagnoses. To identify a tumour agnostic biomarker for rare cancer patients responding to combination immunotherapy we commenced evaluation of baseline and on-treatment collected biospecimen including PBMCs, sera/plasma and tumour tissue for tumor agnostic markers of response including cancer-specific autoantibodies, cytokine levels, PD-L1 status, baseline tumor gene-expression and tumour mutational burden (TMB).