Spinal Chordoma

---

Spinal Chordoma: Symptoms, Causes, Types, Diagnosis, and Treatments

Spinal chordoma is a rare and challenging type of cancer that originates in the bones of the spine. Despite its rarity, spinal chordoma can cause significant health problems due to its location near the spinal cord and nerves. This article provides an in-depth look at spinal chordoma, detailing its symptoms, causes, types, diagnostic procedures, and treatment options. Whether you are a patient, caregiver, or healthcare professional, understanding this disease is vital to managing and overcoming its impacts.

What is Spinal Chordoma?

Spinal chordoma is a malignant (cancerous) tumor that arises from remnants of the notochord — an embryonic precursor to the spinal column. These tumors most commonly develop along the spine, particularly at the base of the skull (clivus), sacrum (tailbone), and vertebral bodies of the spinal column. Although chordomas are slow-growing compared to other cancers, they are locally aggressive, meaning they can invade and damage nearby tissues, especially the spinal cord and nerves.

Symptoms of Spinal Chordoma

One of the challenges with spinal chordoma is that symptoms often develop slowly and are nonspecific early on. Because of its location near the spinal cord and nerve roots, symptoms usually arise from nerve compression and bone destruction. The symptoms vary depending on the tumor’s location along the spine.

Common Symptoms Include:

• Pain: Persistent and worsening pain is often the first symptom. It can be localized to the spine or radiate along nerve pathways. For example, sacral chordomas may cause lower back, buttock, or leg pain.
• Neurological Deficits: As the tumor grows, it can compress the spinal cord or nerve roots causing numbness, tingling, or weakness in the limbs.
• Motor Dysfunction: Weakness or paralysis may develop in the affected limbs, particularly if the tumor compresses motor pathways.
• Bowel and Bladder Dysfunction: Tumors in the sacral region can affect nerves controlling bladder and bowel function, causing incontinence or retention.
• Difficulty Walking: Due to nerve compression or muscle weakness.
• Swelling or Palpable Mass: In some cases, a mass may be felt near the affected vertebrae or sacrum.
• Headache or Vision Problems: For tumors near the base of the skull.

Because these symptoms can mimic other spinal disorders like herniated discs or spinal stenosis, spinal chordoma is often diagnosed late.

Causes of Spinal Chordoma

The exact cause of spinal chordoma is not fully understood, but it originates from notochordal remnants. The notochord is a rod-like structure in the developing embryo that guides spinal development but usually disappears before birth, except in some cells that can give rise to chordoma.

Risk Factors and Theories Include:

• Genetic Mutations: Some studies suggest that mutations or abnormalities in genes such as brachyury (T gene) may play a role. Brachyury is a transcription factor essential for notochord development and is overexpressed in chordoma cells.
• Age and Gender: Spinal chordoma most commonly affects adults aged 40-70 and is slightly more frequent in males.
• Previous Radiation Exposure: There is limited evidence that prior radiation to the spine could increase risk.
• Environmental Factors: No established environmental or lifestyle risk factors have been identified.

Chordoma is not considered hereditary, but ongoing research is examining genetic susceptibility.

Types of Spinal Chordoma

Spinal chordomas are categorized primarily by their location and histological subtype:

Based on Location:

1. Sacral Chordoma
   The most common type, found in the sacrum (lower spine/tailbone area).
   Symptoms: Low back pain, bowel/bladder issues, sciatica.
2. Mobile Spine Chordoma
   Occurs along the vertebrae of the cervical, thoracic, or lumbar spine.
   Symptoms: Neck or back pain, neurological symptoms affecting limbs.
3. Clival Chordoma
   Located at the skull base near the clivus bone, often affecting cranial nerves.
   Symptoms: Headaches, vision changes, cranial nerve palsies.

Histological Subtypes:

• Conventional Chordoma: The most common form, characterized by physaliphorous (bubble-like) cells.
• Chondroid Chordoma: Has features resembling cartilage and tends to have a slightly better prognosis.
• Dedifferentiated Chordoma: Rare, aggressive form with a higher likelihood of metastasis.

Understanding the type and location is critical for treatment planning and prognosis.

Diagnosis of Spinal Chordoma

Diagnosing spinal chordoma involves a combination of clinical evaluation, imaging studies, and biopsy.

Clinical Evaluation

• Medical History and Physical Exam: Detailed history of pain, neurological symptoms, and any bowel/bladder changes.
• Neurological Assessment: Examining motor strength, sensation, reflexes, and gait.

Imaging Studies

Imaging plays a pivotal role in identifying the tumor’s size, location, and relation to surrounding structures.

• X-ray: May show bone destruction or abnormality in vertebrae.
• Magnetic Resonance Imaging (MRI): The gold standard imaging technique for spinal chordoma. MRI provides detailed images of soft tissue involvement and tumor extent, especially around the spinal cord and nerves.
• Computed Tomography (CT) Scan: Useful for evaluating bone destruction and surgical planning.
• Positron Emission Tomography (PET) Scan: Occasionally used to assess metabolic activity or detect metastases.

Biopsy

• A tissue biopsy confirms the diagnosis by microscopic examination.
• Typically performed by a needle biopsy under imaging guidance.
• Immunohistochemical staining for markers like brachyury helps confirm chordoma.

Additional Tests

• Neurological Function Tests: Electromyography (EMG) or nerve conduction studies if needed.
• Blood Tests: No specific markers, but general tests assess patient health.

Early diagnosis can be difficult due to slow symptom progression and the tumor’s rarity, which often leads to delays.

Treatment of Spinal Chordoma

Treatment of spinal chordoma requires a multidisciplinary approach due to the tumor’s complex location and tendency to recur. The primary goals are to remove or reduce the tumor, relieve symptoms, preserve neurological function, and prevent recurrence.

Surgery

Surgery is the mainstay of treatment for spinal chordoma.

• Complete Surgical Resection: The goal is en bloc resection — removing the tumor in one piece with clear margins to reduce recurrence risk.
• Challenges: The proximity to the spinal cord, nerves, and vital structures makes complete removal difficult.
• Sacral Chordomas: May require partial sacrectomy, sometimes affecting bowel and bladder function.
• Reconstruction: After tumor removal, spinal stabilization or reconstruction may be needed.

Radiation Therapy

Radiation therapy is often used as an adjunct to surgery or when surgery is not possible.

• Proton Beam Therapy: A precise form of radiation with less damage to surrounding tissues; preferred due to chordoma’s resistance to conventional radiation.
• Carbon Ion Therapy: An emerging advanced radiation technique with promising results.
• Conventional Radiation: May be used when advanced therapies are unavailable but often less effective.

Radiation can help control residual tumor and delay recurrence.

Chemotherapy

• Chordomas are generally resistant to standard chemotherapy.
• Chemotherapy is rarely used but may be considered in cases of dedifferentiated or metastatic chordoma.
• Targeted therapies and clinical trials are ongoing to find effective drugs.

Targeted Therapy and Experimental Treatments

Research is exploring molecular targets such as brachyury and PDGFR (platelet-derived growth factor receptor) to develop novel treatments.

• Imatinib (Gleevec): A tyrosine kinase inhibitor, sometimes used experimentally.
• Immunotherapy: Trials are investigating immune-based treatments.

Symptom Management and Rehabilitation

• Pain Management: Medications, nerve blocks, and supportive care.
• Physical Therapy: To maintain mobility and strength.
• Bowel and Bladder Care: For sacral tumor patients with dysfunction.
• Psychological Support: Counseling and support groups.

Prognosis and Follow-Up

Spinal chordoma is a slow-growing but locally aggressive tumor with a tendency for local recurrence even after treatment. Long-term prognosis depends on:

• Completeness of tumor resection.
• Tumor location and size.
• Histological subtype.
• Patient’s age and overall health.

Recurrence and Monitoring

• Recurrence is common; hence, regular follow-up with MRI scans is essential.
• Lifelong surveillance is often necessary due to late recurrences.

Conclusion

Spinal chordoma, though rare, poses serious challenges due to its location near critical neural structures and its resistance to conventional therapies. Early recognition of symptoms, accurate diagnosis through imaging and biopsy, and a carefully coordinated multidisciplinary treatment approach are essential to improving outcomes.

Frequently Asked Questions (FAQs) About Spinal Chordoma

For more details keep visiting our Website & Facebook Page.