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Health condition · Clinically reviewed

Chordoma, a rare midline bone tumour that needs specialist care from day one.

Slow-growing, locally destructive and highly treatable when combined en-bloc surgery and proton beam therapy are delivered at a UK specialist centre.

Jump to treatment
A radiographer guides a patient onto the bed of an advanced 3 Tesla MRI scanner in a London imaging suite

Why trust this guide

  • 01

    Clinically reviewed

    Written by our editorial team and reviewed by a registered UK clinician before publication.

  • 02

    Sourced from guidance

    Checked against NICE, BSG bone sarcoma standards and international chordoma consensus you can see at the end.

  • 03

    Current for 2026

    Reflects modern UK practice including proton beam therapy at Christie and UCLH, brachyury-guided diagnosis and targeted trials.

Key facts

Chordoma at a glance.

The essentials, in plain English. What it is, where it grows, and how a UK sarcoma and skull base team approach it.

  • What it is

    A rare malignant bone tumour arising from remnants of the notochord, the embryonic structure that becomes the spine.

  • Where it grows

    Along the midline axial skeleton. About half in the sacrococcygeal region, around 30 per cent at the skull base, and roughly 20 per cent in the mobile spine.

  • How rare

    Roughly one case per million people per year. Most patients are in their 40s to 60s, with a slight male predominance.

  • The diagnostic clue

    Nuclear expression of brachyury (TBXT) on immunohistochemistry is pathognomonic and drives the diagnosis.

  • Behaviour

    Slow-growing but locally destructive, with late distant spread (lung, bone, liver, skin) in around 30 to 40 per cent.

  • Backbone of care

    En-bloc surgical resection with negative margins combined with high-dose proton beam therapy at a specialist centre.

Why this guide matters

A rare tumour, but a well-mapped pathway.

Chordoma is uncommon, so the difference between a local hospital and a specialist chordoma centre is the single biggest factor in outcome.

  • Get to a chordoma MDT early

    RNOH Stanmore, Birmingham Royal Orthopaedic, The Royal Marsden, UCLH, Queen Square, The Christie, Nottingham, Sheffield, Newcastle and Oxford lead UK care.

  • Biopsy is planned, not urgent

    A CT-guided biopsy must be planned by the treating surgeon so the tract can be excised at the definitive operation. Do not biopsy locally first.

  • Surgery plus protons wins

    Complete en-bloc resection combined with high-dose proton beam therapy at Christie Manchester or UCLH London gives the best long-term local control.

How the diagnosis is made

From midline pain to a specialist plan.

The typical UK pathway. Symptoms picked up locally, imaging that raises suspicion, then transfer to a chordoma MDT for biopsy and planning.

  1. 01

    Assessing

    History and midline pain

    Chronic low back or sacral pain, persistent headache with cranial nerve symptoms, or progressive neck pain that does not settle deserves imaging.

  2. 02

    Assessing

    Neurological examination

    Cranial nerves (especially the sixth nerve for diplopia), long-tract signs, bladder and bowel function, and coccygeal tenderness.

  3. 03

    Assessing

    MRI gold standard

    Whole spine MRI shows a T2 hyperintense, heterogeneously enhancing midline mass with soft tissue extension. Essential for surgical and radiotherapy planning.

  4. 04

    Confirming

    CT for bone and calcification

    Defines bone destruction, internal calcification and helps plan reconstruction, particularly at the skull base and sacrum.

  5. 05

    Confirming

    Staging imaging

    CT chest, abdomen and pelvis plus PET-CT to look for lung, bone and visceral metastases before major surgery.

  6. 06

    Confirming

    Biopsy at a sarcoma centre

    CT-guided biopsy planned by the treating team so the tract can be excised at surgery. Never biopsied first at a local hospital.

  7. 07

    Preparing

    Molecular and MDT review

    Brachyury, S100, cytokeratin and INI-1 (SMARCB1) staining, molecular panel, endocrine and neuropsychology baselines, then a specialist chordoma MDT plan.

Typical timeline: weeks between first imaging, specialist transfer and a settled treatment plan.

Symptoms

What chordoma actually feels like.

Symptoms depend on where the tumour sits. Sacrococcygeal disease causes chronic midline pain, skull base disease cranial nerve problems, spinal disease radiculopathy or myelopathy.

  • Chronic sacral or low back pain

    Deep, dull, midline pain that does not respond to usual measures and slowly worsens over months.

  • Coccygeal pain and palpable mass

    Discomfort when sitting, a fullness on rectal examination, or a soft tissue mass low in the buttock.

  • Bladder and bowel dysfunction

    Sacrococcygeal chordoma can compress sacral nerves, causing urinary hesitancy, constipation or saddle numbness.

  • Headache and cranial neuropathy

    Skull base chordoma presents with persistent headache, double vision (sixth nerve palsy), facial numbness or swallowing changes.

  • Visual and hormonal change

    Clival tumours can affect the optic pathways and compress the pituitary, causing visual loss or endocrine symptoms.

  • Neck pain and radiculopathy

    Mobile spine tumours cause axial pain with nerve root or spinal cord signs, sometimes progressive weakness.

  • Slow-growing but relentless

    Symptoms often build over many months before diagnosis, which is why any persistent midline pain warrants imaging.

  • Red flag - late metastasis

    Around a third of patients develop late spread to lung, bone, liver or skin, so lifelong surveillance is standard.

Treatment

How chordoma is treated in the UK.

En-bloc resection with negative margins, high-dose proton beam therapy, and highly selected roles for stereotactic radiosurgery, targeted therapy and trials.

  • En-bloc surgical resection

    The mainstay of curative treatment. Wide, negative-margin removal by a specialist skull base, spine or sacral team, often with reconstruction.

  • Proton beam therapy

    High-dose (70 to 79 Gy equivalent) precision radiotherapy at Christie Manchester or UCLH London that spares brainstem, cord and eyes. See /treatments/proton-beam-therapy/.

  • Stereotactic radiosurgery

    Gamma Knife or CyberKnife for small residual or recurrent disease, or as a boost. See /treatments/gamma-knife-radiosurgery/ and /treatments/cyberknife/.

  • Carbon ion therapy

    An alternative particle therapy used in Europe and Japan for selected chordomas. Not currently available in the UK.

  • IMRT or SBRT

    Advanced photon-based radiotherapy where proton beam is not appropriate or accessible. Lower conformality than protons at critical interfaces.

  • Targeted therapy

    Imatinib (PDGFR), sunitinib, sorafenib and erlotinib have modest activity in selected recurrent or metastatic disease.

  • Tazemetostat for INI-1 loss

    An EZH2 inhibitor with a role in poorly differentiated, INI-1 (SMARCB1) deficient chordoma. Accessed through specialist centres and trials.

  • Immunotherapy and vaccines

    Checkpoint inhibitors and brachyury-targeted vaccines are in clinical trials, offered through the specialist MDT.

What this guide is based on

The sources behind every claim on this page.

UK sarcoma standards, national commissioning policy and international consensus guidance, current at the time of last review.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

A specialist chordoma team knows your imaging, pathology and history and can tell you which parts apply to you. If in doubt, seek a specialist opinion.

  • NICE. Improving outcomes for people with sarcoma (CSG9) and related quality standards.

  • British Sarcoma Group. UK guidelines for the management of bone sarcomas.

  • Chordoma Foundation. Global consensus recommendations on the management of chordoma.

  • NHS England. Highly specialised commissioning for proton beam therapy and skull base surgery.

Red flags

When chordoma needs urgent attention.

Most chordoma follow-up is planned and calm. These are the situations that are not, and where a same-day call to your specialist team is right.

  • Progressive neurological deficit

    New weakness, sensory loss, bladder or bowel change with spinal chordoma needs same-day neurosurgical assessment.

  • New diplopia or cranial signs

    Sudden double vision, facial numbness or swallowing difficulty with a known clival lesion requires urgent MRI and specialist review.

  • Saddle anaesthesia

    Numbness around the perineum with urinary retention suggests cauda equina compromise and is a surgical emergency.

  • CSF leak after surgery

    Clear nasal or wound discharge, positional headache or meningism after skull base surgery must be reported immediately.

  • Infection at reconstruction

    Redness, discharge or systemic upset over a sacral flap or spinal wound needs urgent specialist review.

  • New respiratory symptoms

    Persistent cough, breathlessness or haemoptysis in follow-up may signal lung metastasis and warrants CT.

  • Rapid pain escalation

    A sudden change in pain character or severity at any known chordoma site should prompt urgent restaging.

  • Endocrine collapse

    Severe fatigue, low sodium or hypotension after skull base treatment can reflect pituitary failure and needs endocrine input.

  • Visual deterioration

    Any new visual loss or field defect in a patient with a clival or parasellar chordoma needs urgent ophthalmology and MRI.

Living with it

Rare, serious, and manageable with the right team.

Four things that make the biggest difference over the long term. Specialist care, structured surveillance, real rehabilitation and a support network that gets it.

A quiet reminder

Continuity of specialist care is everything.

Chordoma is a lifelong condition even after successful treatment. Staying under a chordoma MDT gives you the best chance of catching recurrence early.

  1. 01 Specialist care

    Stay under a chordoma MDT

    Outcomes are strongly linked to being treated at a high-volume UK sarcoma and skull base centre, with international collaboration where needed.

  2. 02 Surveillance

    Lifelong MRI follow-up

    Local recurrence and late metastasis can occur years later, so structured MRI and cross-sectional imaging continue for life.

  3. 03 Rehabilitation

    Rehab is part of treatment

    Physiotherapy, continence support, speech and swallow input and pain management make a real difference to day-to-day life.

  4. 04 Support

    You are not alone with this

    The Chordoma Foundation, Bone Cancer Research Trust and Sarcoma UK offer patient support, trial information and second-opinion pathways.

Frequently asked

Everything we get asked about chordoma.

Quick answers on subtypes, brachyury, UK proton beam therapy and long-term follow-up.

  • What exactly is a chordoma?

    Chordoma is a rare malignant bone tumour that develops from remnants of the notochord, the embryonic structure that forms the spine. It grows along the midline axial skeleton, most often in the sacrum and coccyx, at the skull base (clivus) or in the mobile spine.

  • How rare is chordoma and who gets it?

    Chordoma affects roughly one person per million each year. Most patients are diagnosed between their 40s and 60s and it is a little more common in men. A very small number of families carry a duplication of the brachyury (TBXT) gene, which increases risk.

  • What are the main types?

    The classical (conventional) type is the most common. Chondroid chordoma has a slightly more favourable outlook. Dedifferentiated chordoma is rare, high grade and aggressive. Poorly differentiated chordoma is defined by loss of INI-1 (SMARCB1) and is seen in children and young adults. Extra-axial chordoma, arising outside the spine, is very unusual.

  • How is chordoma treated in the UK?

    The mainstay is en-bloc surgical resection with negative margins by a specialist skull base, spine or sacral team, combined with high-dose proton beam therapy at Christie Manchester or UCLH London. Stereotactic radiosurgery, targeted therapy and trials of immunotherapy and vaccines are used in selected situations.

  • Why is proton beam therapy so important?

    Chordoma needs a very high radiation dose to control it, but sits next to the brainstem, spinal cord, optic nerves and other critical structures. Proton beam therapy delivers that dose with a much sharper edge than conventional photon radiotherapy, sparing surrounding tissue and improving the chance of long-term local control.

  • What is the outlook?

    With combined specialist surgery and proton beam therapy, long-term local control is achievable in a significant proportion of patients, particularly at the skull base. Chordoma can recur locally and spread late, so lifelong MRI surveillance and continued care at a specialist centre are essential.

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