Question 1

What is a chordoma?

Accepted Answer

A chordoma is a rare malignant (cancerous) bone tumor that develops from remnants of the notochord - a structure that forms your spine during development as an embryo but normally disappears before birth. In rare cases, some notochord cells persist and can transform into chordoma decades later. Chordomas grow slowly but are locally aggressive, destroying surrounding bone and tissue. They occur almost exclusively in the spine: 50% in sacrum (tailbone area), 35% in skull base (clivus), 15% in mobile spine (cervical/thoracic/lumbar). They're extremely rare: only 300 new cases per year in the USA, roughly 30-40 per year in Australia.

Question 2

Is chordoma cancer?

Accepted Answer

Yes, chordoma is a malignant cancer. However, it's unique because it grows very slowly compared to most cancers. Chordomas typically grow over months to years, not weeks to months like aggressive cancers. They rarely spread (metastasize) to distant sites early - less than 10% have metastases at diagnosis. When spread occurs, it's usually to lungs, liver, or bone. Despite slow growth, chordomas are serious because: 1) They're locally destructive, invading surrounding tissues, 2) They're difficult to remove completely due to location near critical structures, 3) They have high recurrence rates (30-50% recur within 10 years even with best treatment). Five-year survival is 65-70%, 10-year survival about 40%.

Question 3

Why haven't I heard of chordoma before?

Accepted Answer

Chordomas are extremely rare - only about 1 case per million people per year. To put this in perspective: breast cancer affects 1 in 8 women; chordoma affects about 1 in 1,000,000 people. Many doctors will never see a case in their entire career. This rarity means: 1) General awareness is low, 2) Research funding is limited compared to common cancers, 3) Treatment expertise is concentrated in specialized sarcoma centers. The Chordoma Foundation (chordoma.org) is working to increase awareness and fund research.

Question 4

What treatment do I need?

Accepted Answer

Standard treatment is surgery + radiotherapy. Surgery: En bloc resection (removing tumor with surrounding normal tissue margin) at specialized sarcoma center. Goal: achieve negative margins (no tumor cells at edge). If negative margins achieved, radiotherapy may not be needed but often still recommended. If positive margins (tumor cells at edge), radiotherapy is essential. Radiotherapy: High-dose proton beam therapy preferred over conventional radiotherapy (better spares surrounding normal tissue). Given after surgery heals (4-8 weeks post-op), daily treatments for 6-7 weeks. Chemotherapy: NOT effective for chordomas - generally not used. Clinical trials: Ask about targeted therapies or immunotherapies - active research ongoing.

Question 5

Why can't the tumor just be scooped out?

Accepted Answer

Simply 'scooping out' the tumor (intralesional resection) has very high recurrence rates (greater than 90% recur within years). Chordomas have microscopic finger-like projections extending into surrounding tissue that aren't visible to naked eye. If you only remove what you can see, these extensions remain and regrow. En bloc resection (removing tumor with surrounding cuff of normal tissue in one piece, not piecemeal) is required. This often means removing part of sacrum, vertebra, or skull base. The goal is negative margins - confirmed under microscope that no tumor cells at edge of removed specimen. This aggressive surgery is why chordomas require specialized centers.

Medical Disclaimer

Chordoma (Rare Bone Cancer)

📖What is Chordoma (Rare Bone Cancer)?

🔬What Causes It?

⚠️Risk Factors

You may be at higher risk if:

🛡️Prevention