Chordoma
Rare malignant notochordal tumor of the sacrum, clivus, or mobile spine
ICD-10: C41.2 · tumors condition
Chordoma is a rare, locally aggressive, low-to-intermediate grade malignant tumor arising from remnant cells of the primitive notochord — the embryonic structure that guides spine formation and normally regresses. Chordomas occur in the axial skeleton at notochordal remnant sites: the sacrum (50%), clivus at the skull base (35%), and mobile spine — particularly the cervical spine (15%). They are the most common primary bone tumor of the sacrum and spine in adults, though overall incidence is only 1–2 per million per year. Chordomas grow slowly but are locally destructive and carry high local recurrence rates after inadequate resection. They rarely metastasize early but late metastases to lung, bone, and lymph nodes occur in 30–40% of cases. Sacral chordomas present with insidious low sacral or coccygeal pain years before diagnosis; as they enlarge, they compress sacral nerve roots causing bowel, bladder, and sexual dysfunction. MRI and CT demonstrate a destructive sacral mass with a characteristic soft tissue component containing mucoid material and calcifications. En bloc surgical resection with wide (tumor-free) margins is the cornerstone of treatment and is the strongest predictor of local recurrence-free survival. Chordomas are relatively radioresistant to conventional X-ray radiation; high-dose proton beam therapy or carbon ion radiotherapy — which can deliver precision ablative doses without the tolerance limits of adjacent spinal cord and bowel — is the preferred adjuvant treatment and for unresectable lesions.
Anatomy & Pathology
The notochord runs from the clivus at the skull base through the center of each vertebral body to the tip of the coccyx. Vestiges of notochord tissue (ecchordosis physaliphora) are found in a minority of adults but are benign rests, not tumors. Sacrococcygeal chordomas typically arise anterior to the sacrum, expanding the sacral canal and compressing sacral nerve roots that govern lower extremity, bowel, and bladder function.
Symptoms
- Sacrococcygeal or low back pain — dull, progressive, often present for years before diagnosis
- Palpable sacral mass on rectal or pelvic exam
- Bowel dysfunction: constipation, fecal incontinence from sacral nerve compression
- Bladder dysfunction: urinary retention, incontinence
- Sexual dysfunction and perianal numbness from S2–S4 nerve root involvement
- Leg pain or weakness if lumbar or upper sacral nerve roots are compressed
- Cervical or skull base pain with dysphagia or diplopia for clival chordoma
Causes & Risk Factors
- Remnant notochordal cells that persist in the axial skeleton after embryogenesis
- T brachyury gene duplication — a key driver mutation in familial and sporadic chordoma
- Sporadic somatic mutations in notochordal remnants (vast majority of cases have no identifiable risk factor)
Treatment Options
Conservative
- Observation is not recommended for chordoma — diagnosis mandates specialist referral and treatment planning
- Pain management and bowel/bladder support while awaiting definitive surgical planning
- Targeted therapy with imatinib or erlotinib in PDGFR- or EGFR-expressing tumors for unresectable or metastatic disease
Surgical
- En bloc sacrectomy with wide margins — primary treatment for sacral chordoma; margin status is the strongest predictor of recurrence
- Total en bloc spondylectomy (TES) for mobile spine chordoma
- Sacral reconstruction with lumbopelvic fixation after high sacrectomy to restore pelvic ring stability
When to see a spine specialist
Persistent sacrococcygeal or coccyx pain in an adult over 40, particularly without prior trauma, should prompt MRI of the sacrum with gadolinium. Chordoma is frequently misdiagnosed as coccydynia, degenerative disc disease, or disc herniation for years. Any destructive sacral or vertebral lesion on imaging requires referral to a specialty sarcoma or chordoma center before biopsy or resection.
Specialists Who Treat Chordoma
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Search spine specialists →Frequently Asked Questions
What happens if chordoma is not resected with wide margins?
Local recurrence rates after marginal or intralesional resection exceed 50–90% — compared to 20–30% after wide margin resection. Recurrent chordoma is exponentially more difficult to resect due to scar tissue, altered anatomy, and potential for wider tumor spread. The first operation represents the best and often only chance for cure. For this reason, chordoma should be managed at high-volume sarcoma centers with experience in sacral surgery before any resection is attempted. Even core needle biopsy should be planned to avoid contaminating surgical planes.
Why is proton beam therapy used for chordoma instead of conventional radiation?
Chordoma is intrinsically radioresistant — effective tumor control requires high doses (70–80 Gray or more) that exceed the tolerance of adjacent structures (spinal cord, cauda equina, bowel, rectum) when using conventional X-ray photon beams. Proton beam therapy and carbon ion therapy deposit maximum dose at a precise depth (Bragg peak) with minimal exit dose, enabling ablative dosing to the tumor while sparing adjacent critical structures. Proton therapy achieves 5-year local control rates of 60–73% for unresected or residual sacral chordoma.
Will I lose bowel and bladder function after sacral chordoma surgery?
Functional outcomes depend critically on the level of sacral resection. Resection below S3 typically preserves continence; bilateral S3 nerve root preservation maintains bladder and rectal function in most cases. Resection above S2 bilaterally causes permanent bowel and bladder incontinence requiring colostomy and urostomy. Preservation of at least one S3 root provides partial sphincter function. The tradeoff between oncological margin adequacy and functional preservation is the central decision in sacral chordoma surgery.