Childhood Sarcomas
After blood cancers and brain tumors, sarcomas are the most common pediatric tumors. Among these, we distinguish soft tissue sarcomas including rhabdomyosarcomas (RMS, the most common) and bone sarcomas including Ewing sarcomas (ES, the second most common after osteosarcomas). In pediatrics, sarcomas develop at any age and in any part of the body, especially in muscles, nerves, blood vessels, or the fat surrounding bones. In children, approximately 60% of these soft tissue sarcomas are rhabdomyosarcomas (median age: 5 years) and 40% are other types of sarcomas (median age: 9 years). These cancers represent 8% of childhood cancers, about 120 cases per year in France. Ewing sarcoma (ES), which mainly develops in the bones of the pelvis, ribs, femurs, fibulas, and tibias, has a strong invasive potential. Unfortunately, it is not uncommon to see other cancerous foci appear in the body, especially in the lungs, skeleton, and bone marrow at diagnosis.
Unfortunately, despite progress in managing these patients, the survival rate remains very low, especially for patients who do not respond to treatment or present with metastases at diagnosis.
About the Project
Ion channels are structures that allow ions to pass through cell membranes. Several innovative studies on the role of these structures in tumor development are emerging. Aberrant expression of genes encoding these channels has been shown in various types of tumor cells where their original functions are hijacked to promote tumor development. Work led by Dr. Verrechia has shown the involvement of two potassium channels in controlling the proliferation of ES cells. These structures act indirectly by regulating intracellular calcium activity. This highlights the crucial role of calcium in the proliferation of ES cells (publication in preparation).
In this context, Dr. Verrachia’s team proposes to study the role of calcium conductances and intracellular calcium activity in the development of ES and RMS.
These studies should ultimately help define new therapeutic strategies in rhabdomyosarcomas and Ewing sarcomas to not only inhibit the development of the primary tumor and metastatic development but also to promote chemotherapy response. This seems particularly interesting since a wide range of calcium channel inhibitors already exists, which would facilitate the development of this strategy in the clinic.
Project Summary
- Promoter: Center for Cancer Research and Integrated Immunology Nantes – Angers
- Principal Investigator: Dr. Franck Verrechia
- Program Duration: June 2023 – June 2025
- Countries involved: France
- Funding from Imagine for Margo: €60,000
This trial was co-funded by the Rallye du Cœur de Nantes 2023 and was selected as part of the SFCE call for projects in 2022.
