Looking for the perfect trick to differentiate kids with Ewing Sarcoma!

This project was selected in the framework of our fourth European call: FIGHT KIDS CANCER 2023. It is jointly financed by Imagine for Margo, Kriibskrank Kanner Fondatioun, CRIS Cancer Foundation, KiKa and KickCancer.

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Ewing's sarcoma is a malignant solid tumour that most often affects young patients (80% of patients are under 20 years old).

The standard treatment for Ewing's sarcoma includes chemotherapy and local treatment with radiotherapy or surgery when possible. Despite this heavy treatment, the chances of survival for these young patients, when their disease is metastatic at diagnosis, amount to 30% only and half of the relapses occur already while in treatment. Over the last decades, no new effective drugs have been introduced in the frontline treatment. It is high-time we try to improve the chances of survival for those patients.

In the years 2010, a new treatment against a specific target called IGF-1R, which is pre-dominantly present in Ewing’s sarcomas, showed promising results in some patients. However, we have not been able so far to identify which patients would really benefit from this drug.

This project will study samples from patients included in a closed clinical trial where an IGF-1R inhibitor was tested to identify a biomarker that could tell apart patients that re-sponded from those who did not respond.

Besides, a new clinical trial will open shortly in the framework of ESmart where a new antibody called ‘Istiratumab’ will be tested that can target not only IGF-1R but also ERBB3 (a “bis-specific” antibody). This clinical trial will be open to young patients with Ewing’s sarcoma or other tumours with a potential implication of IGF-1R.

The researchers will perform biological studies with fresh tissue of patients’ tumours, collected before inclusion in the ESmart clinical trial, and with plasma collected during the study. A large panel of new technologies will be used to screen all biological ele-ments which could be involved in the response or resistance to treatment, with exten-sive next-generation sequencing for study DNA and RNA and very promising tech-niques to study proteins and metabolites (proteomics and metabolomics).

These new technologies will be used to improve our understanding of the role of IGF-1R in the carcinogenesis of Ewing Sarcoma.

The results could lead to an improved selection of patients for the next clinical trials with targeted therapy against IGF-1R and allow the development of new therapeutic strate-gies for patients with resisting or relapsing Ewing sarcomas.