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Clival chordomas are rare, locally aggressive, slow-growing malignant tumors that arise from notochordal remnants along the midline of the axial skeleton, with the clivus being the most common cranial site.
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Despite being histologically low-grade, they tend to recur locally and invade adjacent critical structures.

Sagittal illustration demonstrating the three divisions of the clivus. The upper clivus extends from the superior aspect of the sella turcica to Dorello’s canal. The mid clivus extends from the inferior border of the sphenoidal sella to the level of the choana. The lower clivus is also known as the “nasopharyngeal clivus” and extends to the craniocervical junction, containing osseous and ligamentous structures.
Soule E, Baig S, Fiester P, et al. Current Management and Image Review of Skull Base Chordoma: What the Radiologist Needs to Know. Journal of Clinical Imaging Science. 2021;11:46. doi:https://doi.org/10.25259/jcis_139_2021

Oblique coronal illustration with the surgical divisions of the clivus in silhouette and its anatomic position relative to the brainstem, posterior circulation arterial vasculature and Cranial nerves.
Soule E, Baig S, Fiester P, et al. Current Management and Image Review of Skull Base Chordoma: What the Radiologist Needs to Know. Journal of Clinical Imaging Science. 2021;11:46. doi:https://doi.org/10.25259/jcis_139_2021
| Feature | Description |
|---|---|
| Age group | 30–60 years (mean ~40–50 years) |
| Sex predilection | Slight male > female |
| Pediatric cases | Rare but more aggressive |
| Feature | Description |
|---|---|
| Embryological origin | Derived from notochordal remnants |
| Common location | Clivus (35–40% of chordomas), followed by sacrococcygeal spine |
| Genetic profile | Involves brachyury (T gene) expression—a notochordal marker |
| Growth pattern | Expansile, infiltrative along bony and soft tissue planes |
Histology
| Component | Description |
|---|---|
| Cell type | Physaliphorous cells: bubbly cytoplasm, central nuclei |
| Matrix | Myxoid background |
| IHC markers | Brachyury⁺, cytokeratin⁺, EMA⁺, S100⁺ |
| Symptom | Mechanism |
|---|---|
| Headache | Mass effect, dural stretching |
| Diplopia | Involvement of CN VI (abducens) or other cranial nerves |
| Facial numbness or weakness | CN V or CN VII involvement |
| Dysphagia / dysarthria | CN IX–XII involvement (lower clival spread) |
| Ataxia / weakness | Brainstem compression |
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Clival chordomas often cross midline and may invade sphenoid sinus, cavernous sinus, and nasopharynx.
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