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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.

Anatomy


![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](attachment:1bb82313-1bec-40f4-8fd5-95299fece98d:JCIS-11-46-g004.png)

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](attachment:b3316fbb-61c9-442f-a5e2-5b8efecf0f67:JCIS-11-46-g005.png)

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

Epidemiology


Feature Description
Age group 30–60 years (mean ~40–50 years)
Sex predilection Slight male > female
Pediatric cases Rare but more aggressive

Pathogenesis


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⁺

Clinical presentation


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

Radiology


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Clival chordomas often cross midline and may invade sphenoid sinus, cavernous sinus, and nasopharynx.

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