Epigenetics for rhabdoid tumours

This project was selected in the framework of our European call: FIGHT KIDS CANCER 2020. It is jointly financed by Imagine for Margo, Kriibskrank Kanner Fondatioun and KickCancer.

It is thanks to your participation in RUN TO KICK 2020 that this innovative project is funded!

Discover the project's video.

Rhabdoid tumours are rare aggressive cancers affecting young children. They mainly occur in the brain, but also in the kidney, liver, ...
Survival rate for the patients with this tumour is under 50% and those surviving will have to go on with their life with oftentimes severe long-term side effects caused by their treatment.

These tumours are characterised by the loss of one unique gene, called SMARCB1. This gene acts to remodel the chromatin and modifies the way the DNA strand is packaged in the cell nuclei. Since there is no other gene defect, SMARCB1 constitutes the only direct targetable gene in this disease.

SMARCB1 is gifted with a very much “epi-genetic” character. Therefore, one major field of research for further therapeutic development concerns drugs that also target epigenetic actors. Epigenetics is the science which explains gene coding: it allows us to understand why cells with the same genetic code have different functions inside someone’s body. Think about a very long book from which some words would be highlighted in different colours to create several alternative plots.

This project will focus on the role of one epigenetic drug in rhabdoid tumours, inhibiting the epigenetic protein “EZH2”, which inhibition has shown some preliminary effects in the clinics.

The impact of the drug on rhabdoid tumours will be analysed at the cell level when grafted in mice. The impact of this drug on the immune environment will also be studied.

This project aims at identifying new treatment options for children with a rhabdoid tumour. As a result, the project will benefit all children (Belgian, European and beyond!) if the study’s conclusions are encouraging.