Imatinib treatment for Chordoma

Imatinib is a form of ‘targeted therapy’ used to block tumour growth in some specific types of cancer. Protein tyrosine kinases represent a group of approximately 90 enzymes which occur normally in the body. These enzymes can be over-active in some cancers and this can contribute to the uncontrolled growth of a tumour. Blocking the activity of these enzymes with drugs known as tyrosine kinase inhibitors can control tumour growth.

The tyrosine kinase known as β receptor of platelet‐derived growth factor (PDGFRB) normally controls the growth and division of cells, particularly of blood vessels. However, it is over-activated in some cancers including some chordomas. Imatinib is a tyrosine kinase inhibitor that blocks the activity of the PDGFRB enzyme and can sometimes help to block the growth of those chordomas where the enzyme is over-expressed/activated. Some clinical studies have suggested that Imatinib may be useful in treating chordoma by slowing growth of the tumour and prolonging survival. However, patients who were treated in this study had inoperable or advanced disease and therefore more research is required to really understand the benefits of this treatment. Another tyrosine kinase inhibitor, Afatinib, which targets the Epidermal Growth Factor receptor (EGFR), is in a clinical trial at the moment.

For any of these tyrosine kinase inhibitors to be effective in treating chordoma, a sample from the tumour needs to be tested by the pathologist to ensure that the target protein, such as platelet‐derived growth factor or Epidermal Growth Factor, is produced in the tumour. Not all chordomas will produce the target protein and may not respond to the inhibitor.

This information is provided for general guidance only and is not intended to be a substitute for specialist and professional medical advice, diagnosis or treatment. It is crucial to seek the advice of your GP, oncologist or other medical specialist with any questions you may have regarding your concerns and/or medical care. Contact us to find out more.

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There is little funding available for research into this relentless bone cancer mainly because it is relatively rare, occurring in only 1 in 800,000 of the population. Without a focused approach to raising money for research into chordoma, little will change quickly.

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