Chordoma

Chordoma: A Rare but Serious Type of Bone Cancer

Chordoma is a rare and slow-growing type of bone cancer that develops in the spine and skull. Though it’s not very common, chordoma is notoriously difficult to treat due to its proximity to critical structures in the nervous system. It arises from remnants of the notochord — a structure that guides the development of the spinal column in the embryo — which normally disappears before birth. However, in rare cases, these remnants persist and give rise to chordomas.

This article provides an in-depth overview of chordoma, including its symptoms, causes, types, diagnosis, and treatment options.

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What is Chordoma?

Chordoma is a malignant tumor that typically occurs in the bones of the spine and the base of the skull. Despite its slow growth rate, it is considered a cancer due to its invasive nature and potential to metastasize (spread) to other parts of the body. Chordomas are usually found in adults between the ages of 40 and 70, though they can also occur in children and young adults, albeit rarely.

Although chordomas account for only 1% to 4% of all bone cancers, their aggressive behavior and location near vital neural structures make them particularly challenging to manage.

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Symptoms of Chordoma

Symptoms of chordoma depend on its location — whether at the base of the skull (clival chordoma), in the spine, or in the sacrum (tailbone). Because chordomas grow slowly, symptoms may take months or even years to become noticeable.

Common Symptoms Include:

1. Persistent Pain
   • Most patients experience dull, aching pain that worsens over time.
   • Pain may be localized in the lower back, neck, or head, depending on the tumor’s location.
2. Nerve Compression
   • Chordomas often press on nearby nerves, leading to numbness, tingling, or weakness.
   • This can result in sciatic-like symptoms if the sacrum is involved.
3. Headaches
   • Clival chordomas (base of skull) can cause severe, recurrent headaches due to pressure on cranial nerves or brainstem.
4. Vision Problems
   • Tumors near the skull base may compress the optic nerves, leading to blurred or double vision.
5. Swallowing Difficulties (Dysphagia)
   • If the tumor affects the cranial nerves, patients may have difficulty swallowing or speaking.
6. Incontinence
   • Sacral chordomas may affect bladder and bowel control due to pressure on the spinal cord.
7. Spinal Deformity
   • Tumors in the vertebrae may cause misalignment or visible deformities.

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Causes of Chordoma

The exact cause of chordoma remains unclear. However, researchers believe that it originates from remnants of the notochord, a primitive structure present during embryonic development that helps in forming the spine.

Risk Factors Include:

1. Genetic Mutations
   • Some studies indicate mutations in the T (brachyury) gene, a key factor in notochord development, may play a role in chordoma formation.
2. Familial Chordoma
   • Though rare, chordoma can run in families, particularly those with a duplication of the T gene.
3. Male Sex
   • Chordoma is more common in men than in women, for reasons not entirely understood.
4. Age
   • Most cases are diagnosed in middle-aged or older adults.

There is no known link between lifestyle factors such as smoking, diet, or environmental exposures and the development of chordoma.

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Types of Chordoma

Chordomas are generally classified based on their location in the body and histological (cellular) characteristics.

Based on Location:

1. Clival Chordoma
   • Occurs at the base of the skull.
   • Affects cranial nerves and brainstem.
   • Represents about 35% of chordomas.
2. Spinal Chordoma
   • Involves the cervical, thoracic, or lumbar spine.
   • Represents about 15% of cases.
3. Sacral Chordoma
   • Affects the sacrum and coccyx (tailbone).
   • Most common type, representing about 50% of chordomas.

Based on Histology:

1. Conventional (Classic) Chordoma
   • Most common type.
   • Characterized by physaliphorous cells (bubbly-looking cells under a microscope).
2. Chondroid Chordoma
   • A mix between chordoma and chondrosarcoma (cartilage tumor).
   • More common in the skull base and may have a better prognosis.
3. Dedifferentiated Chordoma
   • Extremely rare and aggressive.
   • Contains high-grade sarcomatous components.
   • Has a poorer prognosis and higher metastasis rate.

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Diagnosis of Chordoma

Diagnosing chordoma can be challenging due to its rarity and slow progression. Symptoms often mimic other spinal or neurological conditions, which can delay diagnosis.

Diagnostic Steps:

1. Medical History and Physical Examination
   • The doctor evaluates symptoms and performs a neurological exam to assess nerve function.
2. Imaging Tests
   • MRI (Magnetic Resonance Imaging): Best imaging tool for evaluating the extent of the tumor and its effect on nearby tissues.
   • CT (Computed Tomography) Scan: Provides detailed images of bone involvement.
   • X-rays: May show bone destruction but are less useful than MRI or CT.
3. Biopsy
   • A definitive diagnosis is made by removing a sample of tumor tissue for analysis.
   • Typically done using a needle guided by imaging or through surgery.
4. Molecular and Genetic Testing
   • Identifies mutations in the T gene and other biomarkers that may influence treatment options.

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Treatment of Chordoma

Treatment for chordoma is complex and requires a multidisciplinary team, often including neurosurgeons, orthopedic surgeons, radiation oncologists, and medical oncologists.

1. Surgery

Goal: Complete removal of the tumor with clear margins.

• En Bloc Resection: Entire tumor is removed in one piece. Preferred method but difficult due to tumor location.
• Skull Base Surgery: May require advanced neurosurgical techniques to access the clival region.
• Spinal and Sacral Surgery: Carried out with the help of orthopedic or neurosurgical teams.

Challenges:

• Complete resection is often difficult due to proximity to critical nerves, arteries, and the brain.
• Incomplete resection increases risk of recurrence.

2. Radiation Therapy

Used as an adjunct to surgery or when surgery is not possible.

• Proton Beam Therapy:
  • Preferred method due to its precision and ability to spare surrounding healthy tissue.
• Stereotactic Radiosurgery (e.g., CyberKnife):
  • Delivers high-dose radiation in a highly focused manner.
• Conventional Radiation:
  • Used less frequently due to its less precise targeting.

Purpose:

• Reduce tumor size preoperatively.
• Kill residual cancer cells postoperatively.
• Palliative use to relieve symptoms when curative treatment is not possible.

3. Targeted Therapy

Research is ongoing to identify effective targeted treatments for chordoma. Some therapies show promise in clinical trials.

• EGFR Inhibitors (e.g., Erlotinib):
  • Chordomas often express epidermal growth factor receptors (EGFR).
• mTOR Inhibitors:
  • Target pathways involved in cell growth and proliferation.

4. Chemotherapy

• Chordomas are generally resistant to standard chemotherapy.
• May be used in dedifferentiated chordomas or in clinical trials.

5. Immunotherapy

• Investigational use of immune checkpoint inhibitors is being studied.
• Still not considered a standard treatment.

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Prognosis and Survival Rates

Chordoma tends to recur even after treatment, making long-term follow-up essential. Prognosis depends on several factors:

Favorable Prognostic Factors:

• Complete surgical resection with clear margins.
• Younger age.
• Chondroid histology (compared to dedifferentiated).
• Use of proton therapy.

Unfavorable Prognostic Factors:

• Incomplete tumor removal.
• Dedifferentiated histology.
• Older age.
• Tumor recurrence.

Survival Rates:

• 5-Year Survival Rate: Approximately 65% to 70%
• 10-Year Survival Rate: Around 40% to 50%

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Recurrence and Follow-Up

Chordomas are notorious for local recurrence. Long-term monitoring is critical for early detection of recurrence.

Follow-Up Plan:

• MRI scans every 3-6 months for the first few years.
• Annual imaging if no recurrence is detected.
• Neurological evaluations to monitor functional status.

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Living with Chordoma

Living with chordoma poses physical and emotional challenges. Since the condition often requires extensive treatment and follow-up, patients benefit from a strong support system and access to specialized care.

Support Strategies:

1. Rehabilitation:
   • Physical therapy and occupational therapy help restore mobility and function.
2. Pain Management:
   • Ongoing pain due to nerve involvement may need medications, injections, or integrative therapies.
3. Psychological Support:
   • Counseling, support groups, and mental health services help patients and families cope with the diagnosis.
4. Nutrition and Wellness:
   • A balanced diet, adequate sleep, and stress management contribute to overall health.
5. Palliative Care:
   • Focuses on improving quality of life, managing symptoms, and supporting patients through advanced stages of disease.

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Chordoma Research and Clinical Trials

Due to the rarity of chordoma, research is conducted through national and international collaborations. Clinical trials offer patients access to novel therapies and provide hope for improved treatment outcomes.

Promising Research Areas:

• Genetic profiling of tumors to guide personalized treatment.
• New targeted therapies that attack specific tumor mechanisms.
• Immunotherapy options to enhance the body’s natural defenses.

How to Find a Clinical Trial:

• Visit websites like clinicaltrials.gov
• Connect with chordoma foundations or research centers.
• Ask your oncologist about trials that may be appropriate for you.

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Conclusion

Chordoma is a rare but serious bone cancer that demands specialized care and long-term monitoring. While it grows slowly, its location near vital neurological structures makes treatment particularly challenging. Advances in surgical techniques, radiation therapy, and molecular research offer hope for better outcomes and improved quality of life.

Early detection and prompt, expert intervention are key. For those living with chordoma, multidisciplinary treatment, regular follow-up, and emotional support can make a meaningful difference in their journey.

Frequently Asked Questions (FAQs) About Chordoma

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