Chordoma — A Rare Tumour of the Skull Base, Spine & Sacrum

What Is a Chordoma?

A chordoma is a rare malignant tumour that grows out of the bone of the spinal column and skull base. It begins in cells left over from the notochord — the flexible, rod-shaped structure that your spine forms around before you are born. In most people these notochordal cells disappear, but small clusters can persist inside the vertebrae, the sacrum, and the clivus at the base of the skull. Decades later, one of those clusters can begin to grow into a chordoma. This is why chordomas appear almost only along the body's midline axis and almost never in the limbs.

Chordoma is genuinely uncommon — roughly one new case per million people each year — which is exactly why a careful, specialist diagnosis matters so much. Because it is rare and grows slowly, it is often mistaken at first for something more ordinary: chronic back pain, a slipped disc, sinus trouble, or a benign bone lesion. It belongs to the broader family of bone sarcomas, and you can see how it sits alongside osteosarcoma, Ewing sarcoma and chondrosarcoma on our types of bone sarcoma guide. For the full picture of how sarcomas are worked up and treated, the sarcoma — overview hub ties every topic together.

Where Does Chordoma Grow? The Three Main Sites

Symptoms of Chordoma — Why It Is Often Missed at First

Chordoma is slow-growing, so its symptoms creep in gradually and depend entirely on where the tumour is and which nearby structure it is pressing on. There is no single "chordoma symptom"; instead there is a pattern of persistent, location-specific complaints that do not settle with the usual treatments. The most useful question to ask is not "is this serious?" but "why has this not gone away?"

Skull Base (Clival) Chordoma

• Persistent headache behind the eyes or at the back of the head that does not respond to usual painkillers.
• Double vision or a squint, caused by pressure on the nerves that move the eyes.
• Facial numbness, difficulty swallowing, or a change in voice as cranial nerves are affected.
• Nasal blockage or a sense of fullness behind the nose that does not clear like ordinary sinusitis.

Sacral & Spinal Chordoma

• Low back or tailbone pain that is constant, often worse when sitting, and lasts for months without a clear injury.
• Sciatica-like pain, numbness or tingling running down one or both legs.
• Changes in bowel or bladder control — constipation, difficulty passing urine, or incontinence — which always need urgent assessment.
• A feeling of fullness or a lump deep in the lower pelvis or buttock.

How Chordoma Is Diagnosed

Because chordoma is rare and mimics commoner problems, getting the diagnosis right — and getting it right before any surgery — is the most important first step. A planned, specialist work-up protects the one chance to remove the tumour cleanly.

MRI — the Key Imaging Test

MRI is the cornerstone of chordoma diagnosis. A chordoma has a fairly characteristic appearance — a lobulated mass that is very bright on certain MRI sequences — and the scan shows exactly how far the tumour extends, which bone it has destroyed, and its precise relationship to the brainstem, spinal cord, sacral nerves and major blood vessels. That map is what the surgeon and radiation oncologist use to decide whether and how the tumour can be removed in one piece. A CT scan is added to show the bony detail and any calcification.

Biopsy — Done Carefully, by the Right Team

A biopsy confirms that the mass is a chordoma and distinguishes it from chondrosarcoma and other lesions, partly with the help of a marker called brachyury that is specific to notochordal tumours. But a chordoma biopsy must be planned with great care: the needle track itself can carry tumour cells, so it has to be placed along a line that the surgeon can later remove together with the tumour. A carelessly placed biopsy can seed the cancer and compromise the entire operation — which is precisely why a chordoma should be biopsied by the same team that will treat it, not anywhere convenient.

Tumour Board Review

Every confirmed chordoma at CION is presented at the multidisciplinary tumour board, where the surgical oncologist, neurosurgeon or spine surgeon, radiation oncologist, radiologist and pathologist agree a single plan together. For a tumour this rare and this delicately placed, no single specialty decides alone.

Chordoma Treatment — Surgery and Radiation Together

Chordoma responds poorly to ordinary chemotherapy, so treatment rests on two pillars: removing the tumour completely with surgery, and using high-dose radiation to control any cells left behind. The balance between them depends on the tumour's location, size, and whether a clean removal is possible.

Because chordoma tends to recur locally, treatment does not end with surgery and radiation. Lifelong surveillance MRI is part of every plan, so that any return is caught early — when it is small and may again be treatable. This long-term partnership between you and the team is as important as the operation itself.

Outlook, Recurrence and Recovery

Chordoma sits in an unusual place between "slow cancer" and "serious cancer." It rarely spreads early to distant organs, and many people live for years and even decades with it. But it is persistent: if it is not removed completely the first time, it tends to grow back in the same place, and each recurrence is harder to treat than the last because the surrounding anatomy has already been operated on or irradiated. This is why the completeness of the first surgery is the strongest factor in long-term outcome — a clean en bloc removal, ideally combined with high-dose radiation, gives the best chance of lasting control.

Recovery depends heavily on where the chordoma was. Sacral surgery can affect bowel, bladder and sexual function, and a rehabilitation programme with physiotherapy is planned from the start. Skull base surgery requires careful protection of vision, swallowing and the cranial nerves. Whatever the site, the aim at CION is not only to clear the cancer but to preserve as much function and quality of life as the anatomy allows — and to support you through a recovery that is often gradual.

Indicative Cost in Hyderabad

Costs are indicative and vary widely with tumour site and complexity. A personalised estimate is provided after your CION consultation. EMI options and cashless support through major TPAs, Aarogyasri, CGHS, ECHS & ESI are available for eligible patients.

Why Patients Choose CION for Chordoma Care

Chordoma is a once-in-a-million tumour that needs a team that plans every step together. Here is why patients across Telangana trust CION.

Talk to a Chordoma Specialist First

With a tumour this rare, the first plan you make is the one that matters most. If you have a suspected or confirmed chordoma — or one that has come back — bring your scans to a team that treats it.