Chordoma: symptoms, diagnosis and treatment

Cancer Research UK explains that chordomas develop from notochord cells, which form early in the womb as the spine develops. During development the notochord cells get replaced by bone, but sometimes small areas of notochord cells remain in adults. Sarcoma UK classifies chordoma as a rare type of bone sarcoma that can happen anywhere along the spine; the name is pronounced "core-dome-ah".

Sarcoma UK says around 54 people are diagnosed with chordoma each year. Half of all chordomas are in the sacrum (the bottom of the spine), 30% are at the base of the skull and the remaining 20% are in other locations in the spine.

Sarcoma UK describes three main types of chordoma, which help specialists plan treatment.

Cancer Research UK says chordomas tend to grow slowly so people often have symptoms for many months before diagnosis. The symptoms depend on where the chordoma is.

Chordomas are very rare, so it is more difficult for doctors to find out what the causes might be, according to Cancer Research UK. They are more common in men than in women and are usually diagnosed between ages 40 and 60. Researchers are still looking into how chordomas develop.

Cancer Research UK says your doctor will examine you and may order blood tests to check your general health. Other tests may include a CT scan, an MRI scan, a PET-CT scan and a biopsy. Sarcoma UK lists MRI, CT, X-ray and bone scan.

Your doctor or clinical nurse specialist should talk you through the tests and what they show.

Cancer Research UK says treatment depends on where the chordoma is and how big it is, and that it is important to treat it as soon as possible, which helps reduce the chance of it coming back. Sarcoma UK says the main treatments are surgery and radiotherapy, and that chemotherapy is not normally used because research shows it does not work well for chordoma unless the cancer has spread.

Surgery is usually the first treatment. Sarcoma UK explains that the surgeon removes the tumour and aims to take a margin of healthy tissue around it; if wide margins cannot be achieved, radiotherapy is used to kill remaining cells. Cancer Research UK also describes debulking surgery, which removes as much of the tumour as possible. For chordoma in the spine, surgery may affect how the bladder, bowel or legs work, and your specialists will explain the risks and any rehabilitation needed.

Radiotherapy uses high-energy beams to destroy cancer cells. It is used after surgery, particularly if the tumour has been partly removed, to lower the risk of coming back. If surgery is not possible, external radiotherapy is the main treatment, to shrink the tumour and relieve symptoms. Cancer Research UK says some people have proton beam therapy. Sarcoma UK says targeted therapy such as imatinib is not a standard treatment for chordoma, although clinical trials are showing positive results.

Cancer Research UK says it is common for chordoma to come back in the place it started or close by (local recurrence). Treatment may include surgery, radiotherapy, a cancer drug such as imatinib, cryotherapy (using extreme cold to destroy cancer cells) or radiofrequency ablation. Sarcoma UK adds that chordoma can sometimes spread to other parts of the body, with the lungs the most likely site, which is why chest X-rays are part of follow-up.

After treatment you have regular appointments. Cancer Research UK says you usually have an examination and are asked how you feel and whether you have had any symptoms, and you have regular MRI scans. Sarcoma UK says check-ups usually continue for a few years and include an examination, a discussion of symptoms, a chest X-ray and an MRI of the original tumour site.

Sarcoma UK also says rehabilitation, such as occupational therapy, physiotherapy, dietetic or orthotic help, can be useful and with chordoma may be useful to begin earlier; ask your clinical team as soon as possible if you feel you need it and have not been referred.

Cancer Research UK points out that coping with a rare cancer can be especially difficult, and names Chordoma UK, the Bone Cancer Research Trust and the Brain and Spine Foundation among organisations that offer information and support. It also notes that there may be fewer clinical trials for rare cancers because it is hard to recruit enough patients, and suggests asking your specialist whether a trial is suitable.

On the NHS, chordoma is usually diagnosed after scans arranged following symptoms such as persistent pain, neurological changes or vision problems, and care is led by specialist bone and spine or skull base teams. Some people seek a private specialist opinion for a second view. Whichever route you use, your doctor or clinical nurse specialist should talk you through all possible treatment options.