Osteosarcomas in Children
Osteosarcoma is the most common malignant bone tumor or bone cancer (tumors originating in the bone) in adolescents and young adults. Osteosarcoma affects about 100 to 150 new patients per year in France, occurring in young people aged 10 to 25 years and affects boys more (1 girl affected for every 1.4 boys).
Localized osteosarcomas are confined to the bone, the site of the original tumor development (mainly the femur, around the knee, tibia). In 10 to 20% of cases, metastases are present at diagnosis. These metastases are located in 85 to 90% of cases in the lungs, and others later occur in another bone.
For more information on osteosarcomas and our projects to better treat this disease: link to osteosarcoma article.
About the Project
Osteosarcoma (OTS) has a cure rate of 70%. However, new treatments are still needed for patients who are refractory to chemotherapy or in relapse.
Project Objective: Define and characterize sub-classes of OTS at diagnosis with different genetic and biological profiles, taking into account the tumor cell and the bone and immune microenvironment.
Project Description: This project has made it possible to fully exploit the significant genetic and biological data generated from the collection of tumor, blood, and urine samples prospectively collected during the OS2006 protocol. Each biological (bone remodeling, anti-tumor immunity) or genetic aspect was analyzed with clinical and evolutionary characteristics to answer specific questions. The clinical, biological, and genomic data from the various research teams involved in the protocol were combined into a database to be analyzed integratively to determine subgroups of OTS from diagnosis. This database allows other research teams to evaluate the clinical relevance of their preclinical data.
The goal of this project is to propose new targeted treatment options to patients (depending on the OTS sub-class) and to create a database usable by all researchers working on different aspects of OTS.
Project Follow-Up:
- Creation of the OsteoBioNetwork Database to store, share, and visualize all the biological and genomic data generated by the different research teams on the OS2006 cohort (434 patients, sponsored by UNICANCER).
- These data have been consolidated with others from different sources: genomic data produced by Unit UMR 105 directed by Dr. Nathalie Gaspar and other centers.
- Integration of data into cBioPortal.
- Establishment of a rights management system in the database and integration of cleaned and consolidated biological data into OsteoBioNetwork.
- First demonstration on January 21, 2019, at the premises of Unicancer.
- Launch of the “BoOST-DataS” project, which allows OsteoBioNetwork to be extended throughout France and will enable the collection of data on osteosarcomas, particularly from MAPPYACTS or MICCHADO.
- Two publications:
- Successive Osteosarcoma Relapses after the First Line O2006/Sarcome-09 Trial: What Can We Learn for Further Phase-II Trials? – PubMed (nih.gov)
- ctDNA quantification improves estimation of outcomes in patients with high-grade osteosarcoma: a translational study from the OS2006 trial – PubMed (nih.gov)
Project Summary
- Sponsor: Institut Gustave Roussy
- Principal Investigator: Dr. Nathalie Gaspar
- Program Duration: May 2018 – October 2021
- Countries Involved: France
- Funding by Imagine for Margo: €100,000
