Understanding Chordoma:
Diagnosis, Treatment Options and Why a Specialist Matters
A rare cancer that requires rare expertise. Here’s what patients and families need to know.
Understanding Chordoma:
IN THIS ARTICLE
What is Chordoma?
Chordoma is a rare, slow-growing cancer that develops from remnants of tissue formed early on when a baby’s spine is developing in the womb(left over from early foetal development). In the skull base, chordomas most commonly arise at the very base of the skull. They can also develop along the spine or at the bottom of the spine (Sacrum).
Despite growing slowly, chordomas are locally aggressive meaning they tend to push into and erode the structures around them as they expand. They rarely spread to other parts of the body, but their location makes them genuinely difficult to treat.
Around 300 new cases of chordoma are diagnosed in the UK each year. It can occur at any age, but is most common in adults between 40 and 70.
Symptoms to Be Aware Of
Because chordomas grow slowly, symptoms can develop gradually over months or even years before a diagnosis is made. The symptoms vary depending on where in the skull base or spine the tumour is located.
For skull base chordomas, the most common symptoms include:
- Persistent headaches, often at the back of the head
- Double vision or other changes in vision
- Difficulty swallowing
- Facial numbness or weakness
- Neck pain
- A gradual change in the voice
These symptoms overlap with many more common and less serious conditions, which is one of the reasons chordoma can take time to identify. If symptoms are persistent and not resolving with usual treatment, it is worth asking your GP for further investigation.
How is Chordoma Diagnosed?
Diagnosis typically begins with imaging. An MRI scan is the most useful tool for identifying a chordoma and understanding its relationship to the surrounding structures. A CT scan or a PET-CT scan is often also used.
Once imaging suggests a chordoma, a biopsy is usually required to confirm the diagnosis. Because of the location of skull base chordomas, biopsy can be technically demanding and is best performed at a specialist centre with experience in this tumour type. The pathology report from the biopsy will confirm the diagnosis.
Treatment Options
Chordoma is treated primarily with a combination of surgery and radiotherapy. The specific approach depends on the size of the tumour, its exact location, and the extent to which it has grown into surrounding structures.
Surgery
Where possible, surgeons aim to remove as much of the tumour as safely achievable. Because skull base chordomas are adjacent to critical structures such as the brainstem, cranial nerves, and major blood vessels, complete surgical removal is rarely possible without unacceptable risk which is why radiotherapy plays such a central role in managing this cancer.
Proton Beam Therapy
Proton beam therapy is considered the gold standard radiotherapy treatment for skull base chordomas internationally, and for good reason. The physics of proton therapy allow radiation to be deposited precisely at the tumour, stopping almost entirely beyond it, as well as achieving a higher dose of radiation to the tumour while reducing the radaiation damage to surrounding critical structures.
In the UK, proton beam therapy is available privately at Proton International London where Dr Ram often practices.
Conventional Radiotherapy
Where proton beam therapy is not available or appropriate, high-precision conventional radiotherapy techniques such as stereotactic radiotherapy can also be used. These are significantly more precise than standard radiotherapy and can achieve good results in carefully selected patients.
Why a Specialist Matters
Because chordoma is so rare, many oncologists will see only a handful of cases over an entire career. The decisions involved require a depth of experience that simply cannot be built on a small caseload.
The evidence is very clear suggesting clinical outcomes for chordoma patients are better at specialist centres with high case volumes and direct access to proton beam therapy. Patients treated at experienced centres achieve better local tumour control, fewer complications, and better long-term quality of life.
Dr Ram has over 14 years of experience in proton beam therapy and has treated chordoma patients at Proton International London, as well as training at specialist proton centres including , University of Florida Proton Institute, USA, , Proton Therapy Centre in Prague, Paul Scherrer Institute, Switzerland and University of Pennsylvania,USA. He is one of a very small number of consultants in the UK with this specific combination of experience.
Seeking a Second Opinion
If you have been diagnosed with chordoma and are not certain that you are being seen by a specialist in this tumour type, a second opinion is strongly advisable.
A second opinion with Dr Ram will include a detailed review of your imaging and pathology, a clear view on whether your proposed treatment plan is appropriate, and an honest assessment of whether proton beam therapy is the right approach for your situation.
Consultations are available in Southampton, Windsor, and London, and virtually for patients who cannot travel. Read more about the process in Getting a Private Cancer Second Opinion: A Step-by-Step Guide, or contact his medical secretary Ann Clay on +44 23 8120 6598 to arrange an appointment.
Conclusion
Chordoma is rare, slow-growing, cancer and genuinely complex to treat. The good news is that with the right specialist and the right treatment approach, including proton beam therapy where appropriate, good outcomes are achievable.
The most important step is making sure you are seen by a clinician with real, specific experience in this cancer type. If you have any doubt about whether that is the case, it is always worth asking.
Explore the conditions Dr Ram treats, learn more about proton beam therapy, or get in touch to arrange a consultation.
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My approach combines advanced clinical expertise with compassion, honesty, and clear communication. Patients consistently describe my calm, approachable manner and my commitment to explaining every stage of treatment with care and to being there throughout their cancer journey.
Whether you are newly diagnosed with head and neck cancer, exploring proton beam therapy, or seeking private oncology appointments in London, I offer expert, personalised cancer care focused on precision, evidence, and your long-term wellbeing.
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