What is a Chordoma?
Chordoma is a rare cancer that develops from leftover notochord cells. The notochord is an early embryonic structure that helps guide development of the spine and surrounding tissues. Although these cells usually disappear after birth, some may persist and later give rise to chordoma.
Chordoma usually grows slowly, but it is considered a malignant tumour because it can invade nearby structures, recur after treatment, and sometimes spread to other parts of the body. As notochord cells contribute to the formation of the backbone and tend to persist near the base of the skull (the clivus) and the lower spine (the sacrum), chordomas most commonly arise in these regions, although they can develop anywhere along the spine.
Whom does Chordoma affect?
Chordoma most commonly occurs in adults between 50 and 60 years of age. As chordoma usually grows slowly, symptoms may develop gradually over time, which can delay diagnosis.
Chordoma is rare in children and adolescents, with paediatric cases accounting for only a small proportion of all chordoma diagnoses. In children, chordomas are more likely to develop near the base of the skull rather than in the sacrum. In adults, chordoma is more common in males than females.
Types of Chordoma
Chordomas can develop at the base of the skull (clivus), within the spine, or in the sacrum at the base of the spine. About half of chordomas occur in the sacrum, while others develop at the skull base or within the spine.
Doctors classify chordoma not only by where it develops in the body, but also by how the tumour cells look under a microscope. The World Health Organization (WHO) groups chordoma into three main types: conventional (including chondroid), poorly differentiated, and dedifferentiated. Knowing the type of chordoma is important because each one can behave differently and may require a different treatment approach.
The most common form is conventional chordoma, which includes a subtype known as chondroid chordoma. It usually affects adults and is seen more often in men. This type often develops in the sacrum or skull base. Surgery is usually the main treatment, but sometimes it is not possible to remove the tumour completely because of its location or the patient’s overall health. Radiotherapy is often used after surgery, particularly when complete tumour removal is difficult.
Poorly differentiated chordoma is rare and mostly affects children, with an average age of diagnosis around 10 years. This type tends to grow more aggressively and may spread more easily. Because this form behaves more aggressively, treatment may involve a combination of surgery, radiotherapy, and chemotherapy.
Dedifferentiated chordoma is another rare and more aggressive form that usually affects adults around their late 50s. It often occurs in the lower spine and has a higher chance of spreading to other parts of the body. This subtype can be difficult to treat, and ongoing research is exploring more effective therapies.
Chordoma Symptoms
Because chordoma grows slowly, it often does not cause noticeable symptoms in the early stages. Many people only develop symptoms once the tumour becomes large enough to press on nearby nerves, bones, or other structures. In sacral chordoma, tumour growth may eventually interfere with bowel or bladder function.
Symptoms depend on where the tumour develops along the spine.
Chordoma in the sacrum (lower spine) may cause:
- Persistent lower back pain
- Problems with bladder and/or bowel control
- Nerve-related symptoms (radiculopathy), such as shooting pain, numbness, tingling, or muscle weakness
- Compression of nearby nerves can also affect walking, balance, or leg function.
Chordoma at the base of the skull (clival chordoma) lies close to the brain and can affect nerves and surrounding structures. It may cause:
- Headaches
- Double vision (diplopia)
- Cranial nerve dysfunction, which may cause facial weakness, drooping eyelids, facial numbness, or double vision
- In rare cases, tumours near the pituitary region may affect hormone production.
Because chordomas can grow into nearby tissues, they may also cause additional symptoms depending on their location. Tumours located higher in the spine or near the skull base may affect swallowing or breathing if nearby nerves or structures become compressed. . Sacral tumours may extend into the pelvic area and cause constipation, urinary retention, or deep internal (visceral) pain.
How is Chordoma Diagnosed?
Doctors usually investigate suspected chordoma using imaging scans such as computed tomography (CT) and magnetic resonance imaging (MRI). They often use both because each provides different information. A CT scan shows bone damage and areas of calcification more clearly, while an MRI provides detailed information about the tumour’s size and involvement of nearby tissues. .
Doctors combine imaging findings with a patient’s symptoms and examination results to assess the tumour’s location and impact on surrounding structures.
To confirm the diagnosis and guide treatment planning, doctors also need to examine the tumour at a cellular level. They usually do this by taking a small tissue sample or biopsy and examining it under a microscope. The biopsy helps confirm the diagnosis and identify the chordoma subtype before treatment planning.
However, performing a biopsy requires care, as chordoma cells can sometimes spread along the path of the biopsy needle. To reduce the risk of tumour cells remaining in this area, surgeons may remove the biopsy tract during surgery whenever possible.
Living with Chordoma
Living with chordoma can be physically and emotionally challenging for both patients and their caregivers. Because chordoma can recur after treatment, patients usually need ongoing follow-up with a multidisciplinary team that specialises in chordoma care. Regular monitoring helps doctors detect any recurrence early and begin treatment as soon as possible.
Although chordoma grows slowly, it can invade nearby structures and affect important body functions. These effects can impact physical health, emotional well-being, and daily activities, often increasing the need for caregiver support. Sharing how symptoms affect your day-to-day life and discussing the challenges you and your family face can help your healthcare team tailor care to your needs.
Treatment, particularly surgery and radiotherapy, can sometimes lead to long-term complications depending on the tumour’s location. These may include nerve-related symptoms, reduced mobility, ongoing pain, or difficulties with bladder or bowel function in some patients. These effects can significantly affect quality of life. Rehabilitation, physiotherapy, pain management, and supportive care services can help many people manage these challenges and maintain independence.
Chordoma Treatment and Management Options
Treatment for chordoma usually involves a combination of surgery and radiotherapy. Surgery plays an important role in removing the tumour, but because chordomas often grow near critical nerves and structures, surgery can be complex and may involve a long recovery. Potential risks may include pain, infection, nerve damage, reduced mobility, or weakness depending on the tumour location and extent of surgery.
In some cases where surgery is not possible or carries significant risk, doctors may consider high-dose radiotherapy as the primary treatment approach. Clinical trials are continuing to explore the role of radiotherapy and other emerging therapies in chordoma management.
When surgery is possible, doctors aim to remove as much of the tumour as safely possible while achieving clear margins where feasible. Achieving complete removal lowers the risk of the tumour coming back and improves progression-free survival. Radiotherapy, including techniques such as proton beam therapy, is often used after surgery to help control any remaining tumour cells.
Chordomas can be difficult to treat because they often develop near critical nerves, blood vessels, and the brainstem or spinal cord. Although they usually grow slowly, they tend to recur and are often resistant to conventional chemotherapy.
Advances in surgery, imaging, radiotherapy techniques, and targeted therapies are gradually improving outcomes and expanding treatment options for people living with chordoma.
Chordoma is a rare and often complex tumour that typically requires highly specialised treatment and long-term follow-up.
For carers and families, this can involve navigating specialist care, managing recovery from major surgery, and coping with the risk of recurrence.
The National Advocacy Service can help you navigate healthcare systems, connect with services, and advocate for the care your loved one needs. You can also visit our Support Hub for access to free information and resources to help you and your family following a chordoma diagnosis.
References
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- Wedekind, M. F., Widemann, B. C., & Cote, G. (2021, 2021/08/01/). Chordoma: Current status, problems, and future directions. Current Problems in Cancer, 45(4), 100771. https://doi.org/https://doi.org/10.1016/j.currproblcancer.2021.100771