New AcSé-ESMART treatment arm: Testing a novel bispecific antibody targeting IGF-R1 & HER3 in Ewing’s sarcoma and other pediatric cancers
Childhood Sarcomas
Ewing sarcoma is the second most common malignant bone tumor after osteosarcoma among adolescents and young adults. Ewing sarcoma affects approximately 80 to 100 new patients per year in France. The majority of cases (70%) occur in young people aged 5 to 25 years. However, these tumors can be diagnosed before the age of 5 and after 30 years.
Most Ewing sarcomas develop in bone, but 15% can develop independently of a bone.
About BioESMART- IGF-1R/HER3
Promising results were obtained in 2010 for Ewing sarcoma by targeting the IGF-1R protein, a receptor for the IGF-1 hormone, which is essential for growth during childhood. Today, within the AcSé-ESMART program, also supported by Imagine for Margo (link), we have the opportunity to challenge this pathway again with a new bispecific antibody, Istiratumab, which can target both IGF-1R and HER3. HER3 is also a receptor for another growth factor, EGF, and is associated with multiple cancers.
With around 200 new cases per year in Europe, Ewing sarcoma is a difficult disease to cure, and the cure rate has increased very little in recent decades (73% in 2013). With the development of new technologies, particularly in molecular biology, the goal of this study is to better understand the role of IGF-1R in the carcinogenesis of this disease and to identify the specific patient population that responds to targeted treatment.
To achieve this, a new treatment arm will be included within the European AcSé-ESMART trial, to offer Istiratumab to patients with refractory or relapsed Ewing sarcoma or other tumors involving IGF-1R. This Fight Kids Cancer project will conduct biological studies on fresh tumor tissues from patients collected before inclusion, and plasma samples collected during the study. A wide range of new technologies will be used to examine all biological elements that may be involved in response or resistance to treatment, including next-generation sequencing and very promising techniques to study proteins and metabolites (proteomics and metabolomics). The results obtained could improve the guidance of patients in future clinical trials towards IGF-1R-targeting therapy.
Summary of BioESMART- IGF-1R/HER3
- Coordinating Investigators: Dr. Jordane CHAIX
- Program Duration: January 2024 – January 2026
- Countries Involved: France, Spain, Italy, UK, Denmark
- Fight Kids Cancer Funding: €372,000, including €45,050 from Imagine for Margo and €90,000 from the Imagine for Margo Foundation
