Chordoma is a rare, slow-growing malignant bone tumour that arises from remnants of the notochord, a structure present in the embryo. It occurs at the base of the skull (clivus), in the sacrum at the bottom of the spine, or less often in the mobile spine.
It rarely spreads early, but it invades locally and tends to come back if any is left behind. The first operation, done by a team experienced in this exact tumour, offers the greatest chance of control.
Symptoms
- Skull base: double vision, headache, facial numbness, swallowing or voice change
- Sacrum: deep low back or tailbone pain, worse on sitting
- Constipation, or altered bladder or sexual function
- Numbness in the saddle area or leg weakness
- A lump over the lower spine in advanced cases
- Neck pain or arm symptoms with tumours of the cervical spine
Causes and risk factors
Chordomas develop from notochord cells that persist inside the bones of the spine and skull base. Why they turn malignant is not known. There are no lifestyle risk factors. Very rarely the tumour runs in families, linked to duplication of the TBXT (brachyury) gene, and children with tuberous sclerosis have a slightly increased risk. Most patients are between 40 and 70, though skull base tumours occur in younger people and occasionally in children.
How it is diagnosed
- MRI: Defines the extent in soft tissue, nerves and the brainstem or pelvis.
- CT: Shows bone destruction and is needed for surgical and radiotherapy planning.
- Biopsy with brachyury staining: Confirms the diagnosis. For sacral and spinal tumours the needle track must be planned by the operating surgeon so it can be removed with the tumour.
- Staging scans: CT of the chest and sometimes whole spine MRI check for spread.
Treatment options
- En bloc surgical resection: Removal in one piece with a margin, the goal for sacral and spinal tumours. Higher sacral resections can affect bladder, bowel and sexual function, which must be discussed frankly beforehand.
- Skull base surgery: Usually through the nose with an endoscope, aiming for maximal safe removal since a wide margin is impossible.
- High-dose radiotherapy: Proton or carbon ion therapy, or highly conformal photon techniques, after surgery or as the main treatment when surgery would be too damaging.
- Systemic therapy: Standard chemotherapy is ineffective. Targeted drugs such as imatinib may slow advanced disease, and trials are worth asking about.
- Rehabilitation: Physiotherapy, continence and pain services are part of care after sacral surgery.
When it is urgent
Sudden loss of bladder or bowel control, numbness between the legs, rapidly worsening leg weakness, or new double vision with severe headache need emergency assessment locally.
Travelling to Türkiye for treatment
Because it is rare and the first treatment matters most, seeking a high-volume team is justified, in your own country if one exists or abroad if not. Ask how many chordomas the surgeons treat each year and which form of radiotherapy is available, as proton therapy is not offered everywhere. Sacral surgery may mean three to six weeks away including early rehabilitation. MRI follow-up continues for ten years or more and needs a local oncologist.
Send your reports, scans and a short history and a Clinic-Y coordinator replies within 24 hours with suitable teams and written, all-inclusive proposals side by side. Reviewing your case is free.
Frequently Asked Questions
Is chordoma the same as chondrosarcoma?
No. They look similar on scans at the skull base but are distinct under the microscope and behave differently.
Why can it not be treated with chemotherapy?
Conventional drugs have little effect on these slow-dividing cells. Surgery and radiotherapy are the mainstays.
Should I have a biopsy locally first?
Ideally the biopsy is done or planned by the team that will operate, to avoid seeding tumour along the track.
Can it return years later?
Yes, late recurrence is well recognised, which is why scans continue long term.