Chordoma
A rare, slow-growing tumor that can develop along the skull base or spine and affect nearby bone, nerves, and other critical structures.
Quick Answer
A chordoma is a rare, slow-growing but locally aggressive tumor that arises from remnants of the notochord, an embryonic structure, along the skull base and spine. Although chordomas typically grow slowly, they can invade nearby bone and soft tissue and may recur after treatment. Surgery to remove as much of the tumor as safely possible is often a central part of treatment, with specialized high-dose radiation recommended in some cases. Care requires an experienced skull base and spine team.
Key Takeaways
- Chordomas arise from notochord remnants along the skull base and spine.
- They are rare, slow-growing tumors that can invade nearby structures and recur.
- Maximal safe surgical removal is often a central part of treatment.
- Specialized high-dose radiation, such as proton therapy, may be recommended as part of treatment.
- Long-term imaging surveillance is important because chordomas can recur.
- Care requires an experienced skull base and spine team.
At a Glance
| What it is | Rare tumor from notochord remnants |
|---|---|
| Location | Skull base (clivus), spine, and sacrum |
| Behavior | Slow-growing but locally invasive and prone to recurrence |
| Primary treatment | Maximal safe surgical removal |
| Adjuvant therapy | High-dose specialized radiation (often proton therapy) |
| Care model | Experienced skull base and spine team |
Symptoms
Symptoms depend on location. Skull base chordomas, often at the clivus, can cause headaches, double vision, and other cranial nerve problems as they affect nearby nerves. Spinal and sacral chordomas can cause pain and, depending on level, neurological symptoms such as weakness, numbness, or changes in bladder and bowel function.
Because chordomas grow slowly, symptoms often develop gradually and may be present for some time before diagnosis. The specific symptoms reflect which structures the tumor has begun to involve.
Common Warning Signs
- Persistent headaches (skull base chordoma)
- Double vision or other eye-movement changes
- Neck, back, or tailbone pain
- Weakness or numbness
- Changes in bladder or bowel function
When to Seek Care Promptly
- Rapidly worsening vision or double vision
- Progressive weakness or numbness
- New bladder or bowel changes
- Severe or rapidly worsening pain
- New difficulty swallowing or speaking
Diagnosis
MRI with contrast and CT define the tumor and its invasion of bone and soft tissue, and are essential for planning. CT shows the bony destruction characteristic of chordomas, while MRI shows the tumor’s extent and its relationship to nerves and vessels.
A definitive diagnosis requires tissue examination after biopsy or removal, since imaging can suggest but not confirm a chordoma. Careful planning of any biopsy is important in these tumors and is best done by an experienced team.
Treatment
Treatment for a chordoma is individualized based on the tumor’s location, size, extent, prior treatment, and relationship to nearby critical structures. When feasible, maximal safe surgical removal is a central part of treatment, because the extent of the initial resection can influence long-term tumor control.
Skull base chordomas, particularly those involving the clivus, may be approached endoscopically through the nose or with other specialized skull base techniques. Chordomas involving the spine or sacrum require careful surgical planning based on their location and involvement of surrounding structures.
Because chordomas can recur locally, specialized high-dose radiation may be recommended after surgery or in situations where complete removal is not possible. Proton therapy and other highly focused radiation techniques can allow treatment of the tumor while limiting radiation exposure to nearby critical structures.
At Atlantic Brain and Spine, chordoma treatment is coordinated by an experienced skull base and spine team, with multidisciplinary review to determine the appropriate combination and sequence of surgery, radiation, and long-term surveillance.
The Diagnosis-to-Treatment Pathway
1. Evaluation
History, neurological examination, and imaging help identify and characterize the tumor.
2. Detailed imaging
MRI and CT define the tumor's extent and involvement of bone, soft tissue, nerves, and other nearby structures.
3. Planned biopsy
When needed, tissue diagnosis is obtained with careful planning by an experienced team.
4. Multidisciplinary review
Skull base or spine specialists review the diagnosis and develop an individualized treatment plan.
5. Maximal safe resection
When appropriate, surgery aims to remove as much tumor as safely possible while protecting critical structures.
6. Specialized radiation
High-dose focused radiation, including proton therapy in selected cases, may be recommended to improve local tumor control.
7. Long-term surveillance
Regular imaging monitors for residual, recurrent, or progressive tumor.
Frequently Asked Questions
A chordoma is a rare tumor that develops from remnants of the notochord, a structure present during embryonic development, along the skull base and spine. It is slow-growing but locally aggressive, meaning it invades nearby bone and tissue and can recur, which shapes how it is treated.
Chordomas are malignant tumors, but they grow slowly and rarely spread to distant parts of the body early on. Their main challenge is local invasion and recurrence at the original site, which is why complete surgical removal and specialized radiation are emphasized.
The completeness of the first surgery strongly influences the chance of long-term control, because chordomas are prone to recur locally. Achieving a maximal safe removal at the outset, by an experienced team, gives the best foundation for control, usually combined with radiation.
Proton therapy is a specialized form of high-dose radiation that can deliver a strong dose to the tumor while limiting dose to nearby critical structures. It is often used for chordomas after surgery because these tumors sit near sensitive areas and require high radiation doses to control.
Skull base chordomas, often located at the clivus, are frequently approached endoscopically through the nose or with specialized skull base techniques, chosen to reach the tumor while protecting nearby nerves and vessels. The approach depends on the tumor’s exact location and extent.
Chordomas have a tendency to recur locally, which is why maximal safe removal, specialized radiation, and long-term surveillance imaging are all part of care. Detecting recurrence early allows further treatment. An experienced team improves the chance of durable control.
Specialists Who Treat Chordomas
Your care is provided by Atlantic Brain and Spine’s experienced skull base and spine specialists.
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