The challenge
Gastrointestinal stromal tumours (GISTs) are a rare type of cancer that grow in the digestive system. While some GISTs have common gene mutations, others, known as wild-type GISTs (WT-GISTs), do not, and these are harder to treat.
One group of WT-GISTs, known as PAWS-GIST, are ultra-rare and mostly affect children, teenagers, and young adults.
To develop better treatments, researchers need to test drugs on real tumour cells grown in the lab. However, because PAWS-GIST is so rare, it is very difficult to collect fresh tumour tissue to grow these cells. This has slowed progress in finding new treatments.
How will this project tackle this challenge?
Working with the PAWS-GIST patient group and the National GIST Tissue Bank to collect tumour samples, scientists in Nottingham have managed to successfully grow 12 new GIST cell cultures, including 7 from PAWS-GIST patients.
The team will now validate these and additional new PAW-GIST cell cultures, to check that the cells growing in the lab match the tumours they came from. This is important so that future research using these models gives accurate and reliable results.
They will use techniques like DNA sequencing to carefully compare the cell cultures to the original patient samples. Once they are validated, the researchers will freeze and store these cell lines and make them available to other scientists.
The team will also test existing and experimental drugs on the cell cultures, including some drugs already used for other types of cancer, to see if any could be repurposed to help people with PAWS-GIST.
What this means for people affected by sarcoma
These rare tumours have had very few treatment advances because researchers have lacked the tools to study them properly. This project will provide a much-needed resource for the team and wider scientific community supporting faster, better research, including understanding how tumours work, how they resist current drugs, and what new drugs might help.