This trial is co-funded by the Enfants sans Cancer 2020 race and was selected following the Fight Kids Cancer call for projects that we launched in January 2020.
About Pobcorn
Neuroepithelial tumors are generally benign and rare brain tumors that are part of the glial tumor group. They are usually stable in nature and most commonly occur in children and young adults, often associated with epilepsy seizures. However, there are very rare high-grade forms that include BCOR alterations. These alterations give the tumors distinct molecular characteristics, high aggressiveness, and poor clinical outcomes. The POBCORN project, involving an international team, aims to explore all the tumor mechanisms associated with this mutation and potentially find therapeutic pathways. The objective is to identify the oncogenic driving events of this brain tumor as well as potential therapeutic targets to provide the first translational guidance on how to treat patients affected by this cancer who will be included in future clinical trials.
Project Progress
Researchers involved in this project have studied the genetic and epigenetic profiles of 220 tumor samples. These advanced analyses have identified several molecular targets of interest (FGFR(1/3), IGF1R, PDGFR). Screening studies of active and already authorized molecules are currently underway on cultured cells. Initial candidate molecules have been identified, and their combination has led to a reduction in the production of proteins essential for the survival of these tumor cells. This also resulted in a significant slowdown in cell proliferation. Using mouse models grafted with this tumor will confirm these promising initial results and test their therapeutic efficacy in combination with radiotherapy.
These particularly hopeful works were presented at an international conference in 2024 (3rd Pediatric Tumor Symposium in Vienna) and are the subject of 2 publications currently under submission.
Pobcorn Project Summary
- Coordinating Investigators: Drs. Johannes Gojo, Katharina Bruckner, Marcel Kool
- Program Duration: January 2021 – January 2025
- Countries Involved: Austria, Germany, United States
- Funding: Fight Kids Cancer: €500,000, including funding from Imagine for Margo: €239,448
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