What is Chordoma? Diagnosis and Treatment

Written by Medicover Team and Medically Reviewed by Dr Vikram Kishore Reddy P , Neurologists

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1. Causes 2. Find a Doctor 3. Diagnosis 4. Treatment 5. FAQs

Chordoma is a rare malignant tumor that develops from remnants of the notochord, an embryonic structure involved in the formation of the spine. These remnants can persist in the bones of the skull base and spine, where they may give rise to chordoma later in life. Although chordoma typically grows slowly, it is locally aggressive and can invade nearby tissues or spread to other parts of the body.

What are the Types of Chordoma?

Chordoma is classified according to its location and microscopic characteristics.

Conventional Chordoma

This is the most common form, accounting for approximately 80% of cases. It usually develops in the sacrum or the base of the skull.

Chondroid Chordoma

This subtype contains features of both chordoma and cartilage-producing tumors. It most commonly occurs at the skull base and generally has a slightly better prognosis.

Dedifferentiated Chordoma

This rare and aggressive variant contains areas of high-grade sarcoma and is associated with a poorer prognosis than other types.

What are the Causes of Chordoma?

The exact cause of chordoma remains unknown. Researchers believe that genetic mutations and inherited susceptibility may contribute to its development. Most cases occur sporadically, although rare familial forms have been reported.

What are the Symptoms of Chordoma?

The symptoms of chordoma depend on the location of the tumor and the structures it compresses.

Skull Base Chordoma

Headaches
Double Vision or Vision Loss
Facial Pain or Numbness
Difficulty Swallowing
Hearing Loss

Spine Chordoma

Back or Neck Pain
Numbness or Weakness in the Arms or Legs
Difficulty Walking
Loss of Bladder or Bowel Control

End-Stage Chordoma Symptoms

Advanced chordoma may cause severe pain, progressive neurological deficits, and complications resulting from compression of vital nerves and surrounding structures.

When to See a Doctor for Chordoma?

If you experience persistent back or neck pain, numbness, weakness, headaches, vision changes, or problems with bowel or bladder function, consult a Neurologist. Early diagnosis and treatment can help prevent nerve damage, preserve neurological function, and improve treatment outcomes.

If you have persistent back, neck, or tailbone pain that does not improve with rest or medication.
If you experience numbness, tingling, or weakness in your arms or legs.
If you develop difficulty walking, poor balance, or coordination problems.
If you notice headaches, double vision, facial numbness, or difficulty swallowing.
If you experience changes in bowel or bladder control.
If you have a lump or swelling near the spine or base of the skull.
If your symptoms worsen over time or interfere with your daily activities.
If your symptoms persist or worsen, consult a Neurologist for a comprehensive neurological examination, imaging studies, accurate diagnosis, and a personalized treatment plan.

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How is Chordoma Diagnosed?

Diagnosing chordoma involves imaging studies followed by tissue confirmation through biopsy.

Imaging Studies

MRI (Magnetic Resonance Imaging): Provides detailed images of the tumor and surrounding soft tissues.
CT (Computed Tomography) Scan: Evaluates bone involvement and identifies areas of bone destruction.

Biopsy

A biopsy confirms the diagnosis by examining a tissue sample under a microscope and determining the tumor type.

How is Chordoma Treated?

Treatment usually combines surgery with radiation therapy, while targeted therapies may be considered in selected patients.

Surgery

Surgery aims to remove as much of the tumor as possible while preserving nearby nerves and critical structures.

Radiation Therapy

Radiation therapy, including proton beam therapy, is commonly used after surgery or when surgery is not possible to destroy remaining cancer cells.

Targeted Therapy

Targeted therapies may be considered for advanced or recurrent chordoma, although research into newer treatment options continues.

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How is Chordoma Prevented?

There are currently no proven methods to prevent chordoma because its exact cause remains unknown. Individuals with a family history of chordoma or inherited genetic conditions may benefit from regular medical evaluations and imaging studies for early detection.

What is Living with Chordoma Like?

Living with chordoma often requires ongoing medical care, rehabilitation, and emotional support to maintain quality of life.

Follow-Up Care: Attend regular medical appointments to monitor for recurrence and treatment-related complications.
Pain Management: Work with your healthcare team to manage pain using medications, rehabilitation, and supportive therapies.
Support Networks: Join patient support groups and counseling services for emotional support and practical guidance.