What is Chordoma

TL;DR: Chordoma is a rare, slow-growing type of cancer that arises from remnants of the notochord, a structure present during fetal development, typically occurring along the spine or at the base of the skull. While it is locally aggressive and can recur after treatment, it is distinct from other bone cancers due to its unique cellular origin and growth patterns.

What exactly is a Chordoma?

A chordoma is a rare primary bone tumor that develops in the bones of the skull base, the mobile spine, or the sacrum (the base of the spine). Because these tumors arise from the notochord—a flexible, rod-like structure that helps form the skeleton in an embryo—they are most frequently found along the midline of the body. Unlike many other cancers, a chordoma often grows very slowly, though it can be difficult to treat because it frequently develops near critical structures like the brainstem, spinal cord, and major blood vessels.

How common is Chordoma and who is affected?

Chordoma is an extremely rare condition, with an estimated incidence of approximately 0.08 per 100,000 people per year. It can affect individuals of any age, but it is most commonly diagnosed in adults between the ages of 40 and 70. Men are affected by chordoma at a slightly higher rate than women. While there are currently 34 members in the DiseaseMaps.org community living with this condition, it remains a rare diagnosis that requires specialized, multidisciplinary care.

What are the primary classifications of Chordoma?

Pathologists classify chordoma based on how the cells look under a microscope. These classifications help physicians determine the most appropriate treatment plan:

• Conventional Chordoma: The most common type, characterized by cells that resemble the original notochord tissue.


• Chondroid Chordoma: A variant that shares some features with cartilage tumors, often occurring at the skull base and generally carrying a better prognosis.


• Dedifferentiated Chordoma: A rarer, more aggressive form where the tumor cells lose their original characteristics and grow more rapidly.

Next steps

• Consult with a neuro-oncologist or an orthopedic oncologist who specializes in chordoma.


• Request a referral to a high-volume center that has extensive experience in skull base or spinal tumor surgery.


• Join the DiseaseMaps.org community to connect with other patients and share lived experiences.


• Monitor for new neurological symptoms and maintain a regular schedule of follow-up imaging (MRI).

Medical disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment; always seek the advice of your physician regarding a medical condition.

References

• NIH Genetic and Rare Diseases Information Center (GARD): Chordoma.


• Orphanet: Chordoma (ORPHA:193).


• Chordoma Foundation: Understanding the disease and treatment pathways.


• OMIM (Online Mendelian Inheritance in Man): Clinical summary of chordoma.