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Congress: ECR24
Poster Number: C-15001
Type: EPOS Radiologist (educational)
DOI: 10.26044/ecr2024/C-15001
Authorblock: C. García De Andoin Sojo, E. Onis González, J. J. Gómez, M. d. M. Sarmiento, L. Antón, I. Aza, M. Saenz Aguirre, I. Alonso Sánchez, U. Oiartzabal Elorriaga; Bilbao/ES
Disclosures:
Carlota García De Andoin Sojo: Nothing to disclose
Estíbaliz Onis González: Nothing to disclose
Juan José Gómez: Nothing to disclose
Maria del Mar Sarmiento: Nothing to disclose
Lander Antón: Nothing to disclose
Itziar Aza: Nothing to disclose
Martin Saenz Aguirre: Nothing to disclose
Inés Alonso Sánchez: Nothing to disclose
Udane Oiartzabal Elorriaga: Nothing to disclose
Keywords: Bones, Ear / Nose / Throat, Head and neck, CT, MR, Diagnostic procedure, Cancer, Connective tissue disorders, Infection
Findings and procedure details

The clivus is one of the skull base structures formed by the occipital bone and sphenoid bone at the spheno-occipital synchondrosis. It is wedge-shaped and it extends from the foramen magnum to the dorsum sella.

It can show different anatomical variants such as arrested pneumatization

Fig 1: Arrested pneumatization of the skull base. Axial image of a brain CT (A). Sagittal (B) and axial (C) T1-weighted and axial T2-weighted (D) sequences of a brain MRI. Atypical hypodense and T1-T2 hyperintense fatty bone marrow adjacent to the non-pneumatized sphenoid sinus and the clivus.
of the clivus and others derived from the notochord during the developmental period. Those are important in the surgery of the skull base and have the potential to spread infections.

Fossa Navicularis (FN)

FN is a wider-than-deeper notch-like or rounded defect located on the ventral and inferior surface of the basioccipital portion of the clivus. It is postulated that it originates from a notochordal remnant or the expansion of remnant emissary veins.

Fig 2: Fossa Navicularis. Sagittal and coronal images of a brain CT showing FN (arrow).

Canalis Basilaris Medianus (CBM)

CBM is a rare channel communicating the nasopharynx with the intracranial surface of the basioccipital portion of the clivus. Six types are described, three complete forms and three incomplete forms.

Fig 3: Clasification of Canalis Basilaris Medianus (CBM). The three complete forms and two incomplete forms of CBM.
The hypothesis of its origin is the same as that of the FN.
Fig 4: CBM. Axial image of a brain CT. Osseous channel with sclerotic margins in the midline of the basioccipital portion of the clivus (arrow).
Fig 5: CBM. Axial images of different patients' brain CTs showing CMB (arrows).

Craniopharyngeal Canal (CPC)

CPC is a defect of more than 1.5 mm wideness that results in communication between the sella turcica and the nasopharynx. It is supposed to be a remnant of the Rathke´s pouch. There are three types described depending on whether they are solitary or associated with cephaloceles or ectopic adenohypophysis. Wide CPCs are related to transsphenoidal meningoencephaloceles.

The normal bone marrow signal of the clivus is homogeneous and should match the white matter on non-contrast T1 non-fat-saturated images. There are some lesions arising primarily from the osteocartilaginous structure of the clivus that make this normal signal change.

Ecchordosis Physaliphora (EP)

EP or benign notochordal cell tumour (BNCT) is a remnant of the notochord located at the spheno-occipital synchondrosis of the clivus not bigger than 2 cm. It is a T1 hypointense, T2 hyperintense, non-enhancing and no erosive small bony stalk in the intradural space of the prepontine cistern. It is usually discovered incidentally and its radiological features overlap with small chordomas. Therefore, a follow-up MRI should be advised.

Fig 6: Echordosis physaliphora. Sagittal FALIR sequence of a brain MRI (A) and axial image of a brain CT (B). Small well-defined hyperintense lesion (arrows) in the midline occipital portion of the clivus.

Fibrous dysplasia

Fibrous dysplasia is a congenital disorder characterised by the replacement and distortion of normal bone with poorly organised fibrous tissue. It can be isolated or enclosed in a syndrome such as McCune-Albright syndrome, and it also can be monostotic or polyostotic. Craniofacial fibrous dysplasia is one of the most common forms and commonly involves the sphenoid bone, which is related to the clivus.

Lesions are homogeneously dense and sclerotic in the skull bones, expansive, with intact cortex and loss of the corticomedullary differentiation on CT. They are T1 and T2 heterogeneous and show heterogeneous gadolinium enhancement, with no restricted diffusion.

Fig 7: Fibrous dysplasia. Axial (A) and sagittal (B) images of a brain CT. Homogeneously expansive and sclerotic osseous involvement of the skull base affects the clivus.
Fig 8: Cystic fibrous dysplasia. Coronal (A) and sagittal (B) images of a brain CT. T2-weighted sagittal (C) and contrast-enhanced T1-weighted sagittal (D) sequences of a brain MRI. Empty sella turcica and mild erosive changes with cystic areas on the skull base including the clivus.

Paget´s disease

Paget´s disease is a chronic idiopathic alteration of the bone remodelling process. It can affect the skull base bones and make scarce cyst-like changes. Bony CT demonstrates mixed sclerotic and lytic patterns with lytic expansive and cotton wool-appearing areas. On MRI they show T1 hypointensity and heterogeneous contrast enhancement.

Fig 9: Paget´s disease. Sagittal (A) and axial (B) images of a brain CT. Clivus and sphenoid bone alteration consistent with mixed sclerotic and lytic areas.

There are some benign tumours involving the clivus.

Pituitary adenoma

Adenohypophyseal macroadenomas (>10 mm) may extend through the sellar floor and invade different structures, including the clivus. Large adenomas show the “snowman sign” due to the diaphragm sellae and displace laterally the carotid artery. They are heterogeneous with cystic and hemorrhagic areas, hypodense to the brain on CT, T1 iso-hypointense, T2 iso-hyperintense, and show variable restricted diffusion/ADC values and heterogeneous contrast-enhancement on MRI.

Fig 10: Macroprolactinoma. Coronal FLAIR (A), axial T2-weighted (C), axial fat-sat T1 weighted (D) and contrast-enhanced axial fat-sat T1 weighted (E) sequences of a brain MRI. Axial image of a brain CT (B). Erosive heterogeneous mass on the sella turcica that involves de pituitary stalk, displaces laterally the internal carotid arteries and shows mild contrast enhancement.

Petroclival meningioma

Central skull base meningiomas arise from the 2/3 upper of the clivus. They are hyperdense on non-contrast-CT, may have calcifications and show intense and homogeneous contrast-enhancement.  Adjacent bone can show hyperostotic, permeative or sclerotic changes without displacement of the carotid arteries and with no “snowman sign”. In this case, the pituitary gland is preserved. Meningiomas are T1 ant T2 hypo- to isointense to grey matter, may show low or absent signal in calcified areas, rarely have haemorrhagic T1 hyperintense foci, T2 hyperintense CSF-vascular cleft at the periphery of large lesions and prominent homogeneous enhancement and dural tail.

Fig 11: Meningioma. Axial contrast-enhanced T1-weighted fat-sat (A) and sagittal FLAIR (B) sequences of a brain MRI. Homogeneous enhancing and T2 isointense mass with dural tail located on the left side of the cavernous sinus, extending to the clivus and the prepontine cistern.
Fig 12: Meningioma. Axial T2-weighted (A), contrast-enhanced T1-weighted fat-sat (B) sequences of a brain MRI. Axial image of a brain CT (C). Giant meningioma centred in the clival region extending to the masticator space, sphenoid sinuses, left cavernous sinus and enclosing the left internal carotid artery. The lesion is isointense on T2, hyper-enhancing and bone erosive.

Trigeminal schwannoma and Paraganglioma

Trigeminal schwannomas are slow-growing encapsulated benign tumours originating from the Schwann cells. They remodel the bone, are isodense to the brain, T1 hypointense, and T2 hyperintense, show heterogeneous but prominent enhancement, may have cystic areas and can extend from the Meckel cave to the skull base.

Fig 13: Trigeminal V3 Schwanoma. Axial T1-weighted (A), T2- weighted (B) and coronal contrast-enhanced-T1-weighted (C) sequences of a brain MRI. T1 hypointense, T2 hyperintense and contrast-enhancing bilobulated mass in the right Meckel cave and ipsilateral paraclival region (arrows) extending to the maxillary area through the path of the V3 branch of the trigeminal nerve.

Jugular and tympanic paragangliomas grow in the petrous bone and can extend to the paraclival region. They are benign slow-growing neuroendocrine tumours that remodel the adjacent bone and may show a “salt and pepper” pattern in T1, rapid contrast-enhancement and also rapid wash-out.

Fig 14: Jugular paraganglioma. Sagittal T1-weighted (A) and axial contrast-enhanced T1-weighted (B) sequences of a brain MRI. Hyperenhancing left skull-base mass arising the midline and involving the petrous bone and the clivus.

There are also malignant lesions in this area, some primarily of the clivus and others related to surrounding structures.

Chordoma

Chordomas are locally aggressive tumours derived from the notochorda. They have a predilection for the skull base and the sacrococcygeal region. The symptoms caused by them at the skull base originate from the local growth and are usually VI nerve palsy (as it goes through the Dorello canal in the clivus), pain and rhinorrhoea due to a CSF leak.

Even if small chordomas appear as BNCT, big ones appear erosive, lobular, septate, and heterogeneous, T1 hypointense, containing high-signal foci and T2 hyperintensity with T2 hypointense septa. They show a heterogeneous gadolinium-enhancement, with myxoid areas enhancing poorly, and others with variable restricted diffusion.

Fig 15: Chordoma. Sagittal (A) and axial (B) contrast-enhanced fat-sat T1-weighted sequences, axial FLAIR (C) and axial T2-weighted (D) sequences of a brain MRI. Clival midline nodular heterogeneous lesion on T1 and T2-weighted sequences with heterogeneous contrast enhancement extending into the prepontine cistern.
Fig 16: Chordoma. Non-contrast axial images of brain CT (A and C). Axial contrast-enhanced T1-weighted sequence of a brain MRI (B). Clival heterogeneous enhancing lesion extending to the prepontine cistern with mass effect. A transoral CT-guided biopsy was performed (C).

Chondrosarcoma

Chondrosarcomas arise typically lateral to the petroclival synchondrosis and may show on CT “ring-and-arc” pattern of calcifications and chondroid tumour matrix. They are T2 hyperintense with hypointense calcified foci, heterogeneous contrast-enhancement and more extreme values on DWI/ADC sequences than chordomas.

Fig 17: Chondrosarcoma. Coronal (A) and axial (B) images of a brain CT. Axial T2-weighted (A) and contrast-enhanced T1-weighted (D) sequences. T2 hyperintense and heterogeneous enhancing T1 hypointense mass erosive mass centred on the right petroclival synchondrosis and involving the lateral side of the clivus (arrows).
Fig 18: Low-grade chondrosarcoma. Axial contrast-enhanced T1-weighted (A), sagittal FLAIR (B) and axial ASL-perfusion and contrast-enhanced T1-weighted fusion sequences of a brain MRI. Axial image of a brain CT (D). T2 hyperintense and heterogeneous enhancing T1 hypointense mass erosive mass centred on the left petroclival synchondrosis, involving the lateral side of the clivus and the ipsilateral prepontine and cerebellopontine cisterns with no elevation of the ASL and erosion of the bone.

Nasopharyngeal carcinoma

The invasion of the sphenoid sinus and retropharyngeal area gives T3 value to the nasopharyngeal local staging. Higher stages include intracranial extension that can involve clival structures. On CT, invasion of the clivus is seen as bone erosion contiguous to the tumour. MRI shows bone marrow invasion with T1 hypointense, solid contrast-enhancement and restricted diffusion next to the tumour which shows T2 hyperintensity to the muscle, T1 hypointensity and less contrast-enhancement to the mucosa. Perineural invasion through the trigeminal nerve may be observed.

Fig 19: Adenocarcinoma of the cavum. Axial T1-weighted (A) and axial (B) and sagittal (C) contrast-enhanced T1-weighted sequences of a brain MRI. T1 hypointense and homogeneous enhancing mass centred in the nasopharyngeal area extending to the Rosenmuller fossa bilaterally, the clivus and the sphenoid sinuses.

The clivus can be the seat of other malignant lesions in a more global context such as metastases, lymphoma, myeloma, sinus histiocytosis or Rosai Dorfman disease.

Fig 20: Marginal zone lymphoma. Axial contrast-enhanced T1-weighted sequence of a brain MRI (A and B). T1 hypointensity and patchy enhancing (arrow) bone marrow signal alteration.
Fig 21: Sinus histiocytosis/Rosai-Dorfman disease. Axial (A) and sagittal (B) contrast-enhanced T1-weighted sequence of a brain MRI. Skull base meningeal-based mass with nodular enhancement located in the clivus and extending to the sphenoid sinus.

Metastases

Although clival bone metastases are rare, prostate adenocarcinoma, thyroid carcinoma, hepatocarcinoma and breast cancer are the most common origins. Most of the cases are late-stage cancers with diffuse bone involvement and hematogenous spread. CT and MRI features are not specific and they may mimic a primary malignancy.

Fig 22: Metastases of laryngeal carcinoma. Contrast-enhanced axial image of a brain CT. Enhancing masses located in the left auricular and retro-auricular area and the skull base extending to the clivus and the cavum with lytic bone involvement.

Plasmacytoma / Multiple Myeloma

Multiple myeloma and plasmacytoma are malignant proliferations of a single plasma cell clone. CT shows multiple lytic foci without a sclerotic rim, with variable coalescence and extramedullary extensions shown as expansive soft tissue density masses. Plasmacytoma is a single lytic bone lesion associated with a soft tissue mass, being very rare at the skull base. Multiple myeloma lesions are T1 hypointense, T2 hyperintense, homogeneous contrast-enhancement and restricted diffusion.

Fig 23: Multiple myeloma. Axial image of a brain CT (A). Axial contrast-enhanced T1-weighted sequence of a brain MRI (B). Left clival lytic, T1 hypointense and eccentric enhancing (arrow) lesion.

Lesions of inflammatory and infectious origin also affect the clivus and paraclival region. They usually originate in neighbouring structures such as the sphenoid sinus and middle and external ear.

Mucocele

Primary clival mucoceles are extremely rare. They are formed by pneumatization secondary to an obstructed communication with the sphenoid sinus and expansion of cystic masses into other cranial base structures, such as clivus. Mucoceles are T1 variable depending on protein contents, T2 hyperintense, without calcifications and with no contrast-enhancement.

Fig 24: Sphenoid mucocele extending to the clivus. Axial image of a brain CT (A). Axial contrast-enhanced T1-weighted sequence of a brain MRI (B). Non-enhancing cystic right sphenoid sinus mass extending to the anterior right side of the clivus (arrows).

Osteomyelitis

Skull base osteomyelitis is usually related to external malignant otitis or chronic suppurative otitis media. Its diagnosis is crucial to speed up the instauration of antimicrobial therapy. Those patients tend to have comorbidities such as diabetes mellitus, corticosteroids, HIV infection, and chronic rhinosinusitis. Bone erosion, demineralization, contrast-enhancing soft tissue, obliteration of fat planes, involvement of the skull base foramina, and vascular complications can be seen on CT. MRI shows focal or diffuse T1 hypointense clival bone marrow and inflammatory soft tissue with mild restricted diffusion.

Fig 25: Otitis and clival osteomyelitis. Axial T1-weighted fat-sat (A) and contrast-enhanced T1-weighted fat-sat (B) sequences of a brain MRI. T1 hypointense and contrast enhancing bone marrow alteration on the left side of the clivus (arrow) in association with extense soft tissue inflammation centered in the left ear consistent with malignant external otitis.

 

 

GALLERY