What Is Chordoma?

Chordoma is a rare type of tumor that develops in the spine or at the base of the skull. These tumors originate from remnants of the notochord, an embryonic structure that eventually forms the centre of spinal discs.

The most common locations for chordomas include:

• Sacral region (lower back) – Accounting for about one-third to one-half of cases.
• Base of the skull – Making up approximately one-third of cases.

Although chordomas grow slowly, they are considered malignant and can become aggressive over time. They may invade nearby critical structures in the brain and spine, causing pain, nerve damage, and, in severe cases, life-threatening complications.

Symptoms of Chordoma

As a chordoma grows, it can press against nerves, the brain, and the spinal cord, leading to a range of symptoms that vary based on its location. Common symptoms include:

• Persistent pain in the affected area
• Numbness, tingling, or weakness in the limbs
• Loss of bladder or bowel control
• Sexual dysfunction
• Vision disturbances
• Endocrine system disruptions
• Difficulty swallowing
• Presence of a noticeable lump (if the tumor is large)

These symptoms can worsen as the tumor grows, emphasizing the importance of early diagnosis and treatment.

Diagnosing Chordoma

Because chordoma treatment often involves extensive surgery, doctors conduct a thorough diagnostic evaluation before determining the best approach.

Common diagnostic steps include:

• Magnetic resonance imaging (MRI) – Provides detailed images of the tumor and surrounding structures.
• Computed tomography (CT) scan – Helps assess whether the cancer has spread to other areas.
• Biopsy – A needle biopsy may be performed to collect a tissue sample for confirmation. However, because biopsies can sometimes lead to tumor cell spread, surgeons carefully plan the procedure to minimize risks.

Chordoma Treatment

Chordomas can become life-threatening if they metastasize or grow large enough to compress critical structures. Even when they remain localized, they can cause significant nerve damage, potentially leading to permanent disability. Prompt treatment is essential for the best outcomes.

Surgical Removal (En Bloc Resection)

Surgery is the primary treatment for chordoma. The goal is to remove the tumor entirely, including surrounding tissue where cancer cells may have spread. This procedure, known as en bloc resection, offers the best chance for a cure and long-term control of the tumor.

Because chordoma surgery is highly complex, a team of specialists is often involved, including experts in:

• Neurosurgery
• Orthopedic oncology
• Surgical oncology
• Vascular surgery
• Urology
• Plastic surgery
• Anesthesiology

After surgery, patients are monitored in the neurosurgical intensive care unit (NCCU). Once stable, they begin a customized rehabilitation program that may include physical therapy, occupational therapy, and physical medicine to help restore function and mobility.

Radiation Therapy

In some cases, radiation therapy is recommended after surgery to shrink the tumor further or reduce the likelihood of recurrence. A radiation oncologist will develop a tailored plan based on the tumor’s size, location, and aggressiveness.

Long-Term Management of Chordoma

Chordomas have a high risk of recurrence, so ongoing monitoring is essential.

• First-year follow-up – MRI scans are typically performed every three months.
• Long-term monitoring – If no recurrence is detected, the time between MRIs may gradually increase.

Close follow-up care helps detect any regrowth early, allowing for prompt intervention if needed.

While chordomas are rare, their potential to cause serious complications makes early diagnosis and treatment crucial. A combination of surgery, radiation therapy, and long-term monitoring provides the best chance of managing the disease effectively. If you experience persistent pain, numbness, or neurological symptoms, consult a healthcare provider for further evaluation.

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