A great deal has been learned about adult chordoma in the past 10 – 15 years. Much of this knowledge has come from the study of tissue and blood samples from patients with chordoma. The generation of several chordoma cell lines has also been extremely useful to study chordomas. A cell line is a population of genetically identical cancer cells that have been grown in the laboratory after taking a sample of cells directly from a patient. A cell line is referred to as a model of disease and allows researchers to study biological processes unique to one type of cancer.

Tissue and blood samples from chordoma patients have so far helped to:

  • demonstrate the absence of brachyury in dedifferentiated chordomas
  • identify the INI-1 negative (poorly differentiated) chordoma subgroup
  • demonstrate that brachyury is essential for the growth of chordoma cells
  • screen over 1,000 drug compounds that might be effective in the treatment of chordoma
  • determine which of these drug compounds should be taken forward to test in patients in clinical trials
  • build evidence for a clinical trial using drugs against an important cellular protein called Epidermal Growth Factor Receptor (EGFR), which is highly activated in chordoma
  • demonstrate that the brachyury protein is present in virtually all chordomas
  • identify new targets for drug development

Whilst many of the findings about adult chordoma will be relevant to paediatric chordoma, there is still a lot that we don’t yet know about it and it remains understudied.

To advance research into paediatric chordoma, tissue and blood samples are needed from children with chordoma. When a tissue sample is taken from your child it should only be for helping to diagnose and/or treat your child appropriately. Sometimes, there might be some of the sample that is surplus to these requirements – this can be a very valuable resource for research, but doctors and researchers can only use such a sample with the consent of a parent or guardian.

A doctor may approach you before your child is due to have surgery and ask for your consent to take a sample from your child while they’re asleep during surgery. Or they may ask for your consent to take a little extra blood for research purposes when a blood sample is being taken for other purposes. This can seem inappropriate, or even a little offensive, when your minds and hearts are so focussed on getting your child (and indeed your whole family) through an extremely challenging time. It can be very difficult to think about this when you are struggling with so much else. Unfortunately, however, if your consent is not obtained prior to surgery it may mean that some important research into paediatric chordoma cannot be carried out.

Whether or not you provide consent is entirely your choice. We mention it here so that perhaps you can carve out a little bit of time to think through whether you would want to give consent should you be asked – to be forewarned is to be forearmed.

It is the intention of The Drew Barker-Wright Charity to fund vital research into paediatric chordoma so that doctors can learn more about his rare and devastating cancer. So far, we have contributed £30,000 of funding towards Professor Adrienne Flanagans’s project taking place at The Cancer Institute, University College London through The Chordoma Foundation, and another £20,000 directly.

One of the aims of this project is the formation of a UK-wide Chordoma Cohort: as chordoma is so rare it is important to identify all children with chordoma in the UK so that we can learn more about this disease. We hope this will represent the first step towards the initiation of clinical trials of promising new drugs for paediatric chordoma.

Read more about the project The Drew Barker-Wright Charity is helping fund.

To be put in touch with Professor Adrienne Flanagan and her team directly, to make sure they are aware of your child’s diagnosis, please contact their coordinator at The Royal National Orthopaedic Hospital. 

Biobank and Chordoma Coordinator: Tamanah Fayzan.

E: Tamanah.fayzan@nhs.net  T: 0208 909 5347