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Spinal Chordoma

What is Spinal Chordoma?

Spinal chordoma is a rare type of bone cancer, known technically as a primary bone malignancy, that originates from the remnants of the notochord, a flexible rod shaped like a cartilage structure that is important during embryonic development. These tumors are slow-growing and can occur anywhere along the spine but most frequently develop at the skull base, known as the clivus, or the lower spine, specifically the sacrum and coccyx. Despite their slow growth, spinal chordomas can be aggressive and challenging to treat because of their proximity to critical nervous structures.

Symptoms and Diagnosis

Common Symptoms

Spinal chordoma symptoms largely depend on the tumor’s location within the spine. Some of the common symptoms include:

  • Pain: Back pain is the most common symptom. It's often persistent and doesn't improve with rest.
  • Neurological Symptoms: Such as numbness, weakness, or tingling in the limbs, originating from spinal cord or nerve root compression.
  • Bowel & Bladder Dysfunction: Mostly associated with sacral tumors, due to compression on nerves controlling these functions.
  • Headache: If the tumor is located at the skull base.
  • Protrusions or Lumps: Occasionally, a visible mass may be detected, especially in sacral tumors.

Diagnostic Procedures

Diagnosing spinal chordoma involves several steps:

  1. Physical Examination: An initial neurological evaluation to assess symptoms and overall health.
  2. Imaging Tests:
    • MRI (Magnetic Resonance Imaging): Offers detailed images of the spinal cord and surrounding tissues.
    • CT Scans (Computed Tomography): Helpful to evaluate bone destruction.
    • X-rays: Provide a basic view of bone structure but are less detailed than MRI and CT.
  3. Biopsy: A definitive diagnosis typically requires a biopsy, where a tissue sample is taken and examined for cancer cells.

Treatment Options

Surgical Intervention

Surgery is generally the primary treatment for spinal chordomas. The aim is to remove the tumor entirely to prevent recurrence. Surgical options include:

  • En Bloc Resection: This is the preferred method where the entire tumor is removed in one piece, maintaining intact boundaries around it. It's challenging due to the tumor's proximity to vital structures.
  • Partial Resection: When complete removal is not feasible due to risk to critical anatomy, reducing as much of the tumor as possible is considered.

Radiation Therapy

Given the difficulty in completely resecting spinal chordomas, radiation therapy is often employed post-surgery to target residual cancer cells. State-of-the-art options like Proton Beam Therapy and Stereotactic Radiosurgery offer high-precision targeting, minimizing damage to surrounding tissues.

Chemotherapy

Chemotherapy is generally not effective for spinal chordomas. As research progresses, new targeted therapies and immunotherapy options are being explored.

Rehabilitation

Post-treatment, rehabilitation is often essential, focusing on physical recovery and managing potential neurological deficits. Physiotherapy and supportive care play a key role in improving quality of life.

Prognosis and Survival Rates

The prognosis for spinal chordoma patients varies based on several factors:

  • Complete Surgical Resection: The potential for cure increases significantly when the entire tumor can be removed.
  • Tumor Location and Size: Tumors located at the base of the skull are more complex to operate on and have different prognostic outcomes compared to those on the sacrum.
  • Patient's General Health and Age: Overall health also impacts recovery and survival rates.

The five-year survival rate for spinal chordoma ranges from 50-68%, but long-term survival strongly depends on the success of initial treatment.

Living with Spinal Chordoma

Managing Symptoms and Treatment Side Effects

  • Pain Management: Utilizing medications such as NSAIDs, opioids, or nerve blocks.
  • Psychological Support: Given the emotional toll of diagnosis and treatment, psychological support or therapy can be beneficial.
  • Nutrition and Exercise: A balanced diet and suitable exercise regime can facilitate recovery.

Support Systems

Forming a network for emotional, social, and medical support is vital. Organizations such as the Chordoma Foundation offer resources and connection with others affected.

Frequently Asked Questions

Can spinal chordoma recur after treatment?

Yes, recurrence is possible, which necessitates regular follow-ups to monitor for signs of recurrence or metastasis.

Is spinal chordoma hereditary?

There’s no evidence suggesting spinal chordoma is hereditary, though a rare genetic condition called tuberous sclerosis may increase risk.

What research is being conducted?

Much research focuses on genetic mutations linked to chordomas, aiming to develop targeted therapies. Clinical trials are ongoing, examining the efficacy of newer drugs and treatment strategies.

Conclusion

Navigating a spinal chordoma diagnosis involves understanding its implications, options for treatment, and steps for managing life during and after treatment. While challenging, continued research and support systems provide hope and avenues for improving patient outcomes. For further information, discussing with your healthcare provider and exploring resources like the Chordoma Foundation may offer additional guidance and support.