What is Chordoma?
Chordoma is a rare type of cancer that occurs in the bones of the skull base and spine. It arises from remnants of the notochord—a structure found in embryos that helps form the spine. Although the notochord disappears during fetal development, small remnants can remain in the spine and potentially develop into a chordoma later in life.

Chordomas are slow-growing tumors, but they are considered malignant because they can invade nearby tissues and organs, and sometimes spread (metastasize) to other parts of the body. Most chordomas are diagnosed in adults between the ages of 40 and 70, though they can occur at any age.

What Causes Chordoma?
The exact cause of chordoma is not fully understood. It is thought to be linked to leftover notochordal cells that transform into cancer over time. Risk factors are not clearly defined, but some potential contributing factors include:

• Genetic mutations: Some studies suggest that certain inherited or spontaneous gene changes may play a role.
• Family history: Rarely, chordoma can run in families, although most cases occur randomly.
• Tuberous sclerosis: This genetic disorder may slightly increase the risk of developing chordoma in rare instances.

Unlike many cancers, lifestyle factors such as smoking or diet are not known to influence the risk of developing chordoma.

What Are the Symptoms of Chordoma?
Symptoms depend on the location of the tumor, and since chordomas grow slowly, symptoms may take time to appear. Common locations and associated symptoms include:

Skull base (clival chordoma):

• Headaches
• Double vision or other vision changes
• Difficulty swallowing
• Facial numbness or weakness
• Hearing loss

Spinal chordoma (cervical, thoracic, lumbar, or sacral spine):

• Neck or back pain
• Numbness, tingling, or weakness in the arms or legs
• Loss of bowel or bladder control (if the tumor compresses nerves)
• Difficulty walking or balance issues

In general, chordoma symptoms tend to worsen gradually over time, making early diagnosis challenging.

How is Chordoma Diagnosed?
The diagnostic process for chordoma includes:

• Medical history and physical exam: To evaluate symptoms and check for neurological changes.
• Imaging tests:
  • MRI (Magnetic Resonance Imaging): Provides detailed images of soft tissues and is typically the most useful scan.
  • CT (Computed Tomography): Helps assess bone involvement and structural damage.
• Biopsy: A tissue sample is taken from the tumor and analyzed under a microscope to confirm the diagnosis.

Because chordoma can mimic other tumors, it’s essential that diagnosis is confirmed by a pathologist with experience in rare bone cancers.

How is Chordoma Treated?
Chordoma treatment often involves a combination of surgery and radiation therapy. The main goals are to remove as much of the tumor as possible and to prevent it from coming back.

• Surgery: This is the primary treatment. Because chordomas are often located near critical structures like the brainstem, spinal cord, or major blood vessels, complete removal can be challenging.
• Radiation therapy: Used after surgery to target any remaining tumor cells. High-precision radiation types like proton beam therapy or stereotactic radiosurgery are often recommended due to the tumor’s sensitivity and delicate location.
• Targeted therapy: In some advanced cases, targeted drugs may be considered, especially if the tumor cannot be completely removed or recurs.
• Clinical trials: Because chordoma is rare, ongoing research and clinical trials are often exploring new treatments.

What Are the Types of Chordoma?
Chordoma can be classified into a few subtypes:

• Conventional (classic) chordoma: The most common type.
• Chondroid chordoma: Has features similar to cartilage tumors and may have a better prognosis.
• Dedifferentiated chordoma: A rare and aggressive subtype with a higher chance of metastasis and recurrence.

Identifying the subtype helps guide treatment decisions and predict outcomes.

What Are the Risk Factors for Chordoma?
Chordoma is rare, and few risk factors are well-established. However, the following may play a role:

• Age: Most common between 40 and 70 years old.
• Gender: Slightly more common in men.
• Genetic predisposition: Very rarely, certain inherited mutations may increase risk.

There are no clear environmental or lifestyle risk factors for chordoma.

What Are the Complications of Chordoma?
Because chordoma grows near critical structures, it can cause serious complications:

• Nerve damage: Pressure on nerves can lead to chronic pain, weakness, or paralysis.
• Brainstem or spinal cord compression: Can lead to life-threatening complications or permanent neurological damage.
• Difficulty swallowing or breathing: If the tumor is in the upper spine or base of the skull.
• Recurrence: Chordomas frequently come back, even after successful surgery.
• Metastasis: Although slow-growing, chordomas can spread to the lungs, liver, or other bones in some cases.

Regular follow-up care is essential to monitor for recurrence or complications.

How Can Chordoma Be Prevented?
There is currently no known way to prevent chordoma. Since most cases are sporadic and not linked to lifestyle or environmental exposures, prevention strategies are limited. However, for those with a family history or genetic risk, close monitoring and early imaging may be helpful.

What is the Prognosis for Chordoma?
The prognosis for chordoma depends on several factors, including:

• Tumor size and location
• Whether the entire tumor can be removed surgically
• Tumor subtype and grade
• Patient’s overall health

In general, chordomas grow slowly but have a high risk of coming back. With aggressive treatment, some patients may live many years after diagnosis. However, recurrence and metastasis are possible, and long-term management is often needed.