The Chordoma Foundation is a non-profit organisation in the US which focuses solely on improving outcomes for chordoma patients by accelerating research and by helping patients to get the best care possible. Their work has led to some exciting progress in the field of chordoma recently, which we are delighted to share with you.

What is chordoma?

Chordoma is a rare form of primary bone cancer, affecting just 35 people in the UK and Ireland annually, which most frequently arises in the spine or the skull. The varying locations of these tumours can lead to different symptoms when the tumour first presents in patients. This symptom variation alongside the slow-growing nature of the tumour can often make the diagnosis of a chordoma difficult and delay the time it takes for patients to access the correct treatment they need.

The current picture for chordoma

Currently, the main treatment strategy for chordoma patients involves the surgical removal of the tumour followed by radiotherapy. Over recent years, this form of bone cancer has received a lot of research attention and scientists are working hard to develop advanced radiotherapy techniques and targeted treatments in order to improve chordoma treatment.

There is ongoing research into the development of targeted drug therapies, which target a specific molecule that may be overexpressed or mutated in this cancer type and not in healthy cells. Development of targeted drug treatments may help improve survival for chordoma patients and so far molecular targets such as EGFR, PDGFR and VEGF have been investigated. These specific ‘molecular targets’ for targeted drug treatments have the potential to be safer and more specific than conventional chemotherapy agents.

New clinical research

In an exciting development for this form of bone cancer, chordoma patients are now being enrolled in a Phase 1 clinical trial which is studying the safety and effectiveness of an immunotherapy drug called nivolumab, both with and without the addition of stereotactic radiosurgery. Stereotactic radiosurgery (SRS) is an advanced radiotherapy technique used to treat abnormalities and small tumours of the brain with high precision and lower side-effects to healthy tissues surrounding the tumour.

This study is taking place in the US at Johns Hopkins Hospital and Memorial Sloan Kettering Cancer Center and is run by Dr Michael Lim and Dr Yoshiva Yamada in the respective sites. The study aims to harness the body’s own immune system to treat chordoma more effectively and will be open to patients over the age of 15 years with recurrent or metastatic tumours. Clinical trials testing this combination in other tumour types have shown evidence of enhanced activity against the tumour compared with nivolumab or stereotactic radiosurgery used alone and recently, researchers showed that this combination reduces tumour growth and improves survival in a mouse model of chordoma. Taken together, this evidence provides strong rationale for a clinical trial testing nivolumab alone and in combination with stereotactic radiosurgery.

Learn more about this clinical trial, and the treatments being investigated, on The Chordoma Foundation website by clicking here.

New treatment guidelines for chordoma

In February 2015, the first ever clinical guidelines for the diagnosis and treatment of chordoma were published. You can read these guidelines here. These guidelines outline details on factors such as the location of the tumour and varying treatment options to provide a consensus on the most effective care for chordoma patients. These guidelines were initiated during a 2013 meeting supported by The Chordoma Foundation, which brought together experts in chordoma from around the world.

Now, in 2017, a second set of guidelines have been published which detail the best clinical practice for advanced chordomas and chordomas that have returned; known as recurrent chordoma. These guidelines were developed by a group of more than 60 experts in the field from the US, Europe and Japan and detail knowledge on the challenges surrounding a suspected chordoma recurrence and how to treat such recurrence with either curative or palliative intent. You can view these guidelines here.

Together, these two guideline publications set the standard for the best care of chordoma patients, and provide reliable references upon which to base educational initiatives for both professionals and patients.

You can read more about this new set of guidelines and how they were developed on The Chordoma Foundation website, here.