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Comprehensive Review of Intracranial Chordoma¹

¹ From the Departments of Radiology (E.E., E.C.A., R.V.H., J.S.P.) and Neurosurgery (O.A.M.), University of Arkansas for Medical Sciences, 4501 W Markham St, Little Rock, AR 72205. Presented as an education exhibit at the 2001 RSNA scientific assembly. Received December 18, 2002; revision requested February 20, 2003 and received March 26; accepted March 31. Address correspondence to E.E. (e-mail: erenmri@yahoo.com).

View larger version (67K): [in a new window]   Figure 1a.  (a) Three-dimensional CT scan demonstrates the sites of origin of intracranial chordomas: the upper (yellow), middle (red), and lower (green) clivus. (b-d) Sagittal T1-weighted MR images demonstrate involvement of the upper (b), middle (c), and lower (d) clivus.

View larger version (80K): [in a new window]   Figure 1b.  (a) Three-dimensional CT scan demonstrates the sites of origin of intracranial chordomas: the upper (yellow), middle (red), and lower (green) clivus. (b-d) Sagittal T1-weighted MR images demonstrate involvement of the upper (b), middle (c), and lower (d) clivus.

View larger version (94K): [in a new window]   Figure 1c.  (a) Three-dimensional CT scan demonstrates the sites of origin of intracranial chordomas: the upper (yellow), middle (red), and lower (green) clivus. (b-d) Sagittal T1-weighted MR images demonstrate involvement of the upper (b), middle (c), and lower (d) clivus.

View larger version (93K): [in a new window]   Figure 1d.  (a) Three-dimensional CT scan demonstrates the sites of origin of intracranial chordomas: the upper (yellow), middle (red), and lower (green) clivus. (b-d) Sagittal T1-weighted MR images demonstrate involvement of the upper (b), middle (c), and lower (d) clivus.

View larger version (156K): [in a new window]   Figure 2.  Photomicrograph (original magnification, x400; hematoxylin-eosin stain) shows vacuolated cells with intracytoplasmic mucus droplets (physaliphorous appearance) (arrows), a finding that is typical of chordoma.

View larger version (74K): [in a new window]   Figure 3a.  (a) Sagittal reformatted CT scan reveals bone sequestra at the distal end of a lytic clival lesion (arrows). (b) Axial CT scan of the skull base demonstrates the lesion with a clival origin and extension to the prepontine cistern with typical trabecular entrapment (arrow). Dystrophic calcification is also seen (arrowhead).

View larger version (169K): [in a new window]   Figure 3b.  (a) Sagittal reformatted CT scan reveals bone sequestra at the distal end of a lytic clival lesion (arrows). (b) Axial CT scan of the skull base demonstrates the lesion with a clival origin and extension to the prepontine cistern with typical trabecular entrapment (arrow). Dystrophic calcification is also seen (arrowhead).

View larger version (144K): [in a new window]   Figure 4.  CT scan shows a chondroid chordoma in the clivus with intratumoral calcifications (arrow).

View larger version (151K): [in a new window]   Figure 5a.  (a) Axial T1-weighted MR image shows a small, hypointense mass in the right side of the clivus (arrow). (b) Sagittal T1-weighted MR image obtained in a different patient shows a large, hypointense soft-tissue mass that arises from the distal clivus with anterior extension into the nasopharynx (arrows) and extradural extension into the posterior fossa (arrowhead).

View larger version (180K): [in a new window]   Figure 5b.  (a) Axial T1-weighted MR image shows a small, hypointense mass in the right side of the clivus (arrow). (b) Sagittal T1-weighted MR image obtained in a different patient shows a large, hypointense soft-tissue mass that arises from the distal clivus with anterior extension into the nasopharynx (arrows) and extradural extension into the posterior fossa (arrowhead).

View larger version (161K): [in a new window]   Figure 6.  Sagittal T1-weighted MR image shows a retroclival mass (arrows) that has a hyperintense rim and projects posteriorly, a finding that represents highly proteinaceous material or blood products.

View larger version (149K): [in a new window]   Figure 7.  Axial T2-weighted MR image shows an intracranial chordoma with diffuse homogeneous hyperintensity.

View larger version (150K): [in a new window]   Figure 8.  Axial T2-weighted MR image demonstrates a multiseptate, hyperintense mass with extension into the sellar area and left cavernous sinus. The mass also exhibits areas of hypointensity, possibly secondary to calcification, hemorrhage, or a mucus collection.

View larger version (179K): [in a new window]   Figure 9.  Axial T2-weighted MR image shows a septate tumor with a pseudoencapsulated appearance.

View larger version (167K): [in a new window]   Figure 10a.  (a) Axial unenhanced T1-weighted MR image shows an isointense mass along the right side of the clivus and petrous apex. (b) Axial contrast-enhanced T1-weighted MR image shows the mass with marked enhancement.

View larger version (163K): [in a new window]   Figure 10b.  (a) Axial unenhanced T1-weighted MR image shows an isointense mass along the right side of the clivus and petrous apex. (b) Axial contrast-enhanced T1-weighted MR image shows the mass with marked enhancement.

View larger version (168K): [in a new window]   Figure 11a.  (a) Sagittal unenhanced T1-weighted MR image shows a large, isointense soft-tissue mass in the distal clivus (arrows). (b) On a sagittal contrast-enhanced T1-weighted MR image, the mass exhibits little or no enhancement (arrows). Note the normal enhancement of the nasal, nasopharyngeal, and palatal mucosa.

View larger version (171K): [in a new window]   Figure 11b.  (a) Sagittal unenhanced T1-weighted MR image shows a large, isointense soft-tissue mass in the distal clivus (arrows). (b) On a sagittal contrast-enhanced T1-weighted MR image, the mass exhibits little or no enhancement (arrows). Note the normal enhancement of the nasal, nasopharyngeal, and palatal mucosa.

View larger version (183K): [in a new window]   Figure 12.  Axial contrast-enhanced T1-weighted MR image shows a large midclival mass with variable enhancement (honeycomb appearance) and extension to the sellar area and adjacent cavernous sinuses. Note the lateral displacement of the right cavernous internal carotid artery (arrow).

View larger version (152K): [in a new window]   Figure 13a.  (a) Sagittal T1-weighted MR image demonstrates posterior and superior elevation of the right cavernous internal carotid artery (arrows). (b) Composite MR angiogram obtained in a different patient shows posterior displacement of the right posterior cerebral artery (arrowhead) and lateral displacement of the right cavernous carotid artery (arrow). (c) Coronal T1-weighted MR image obtained in a third patient shows a tumor with extension to the right cavernous sinus and concomitant displacement and partial encasement of the right cavernous internal carotid artery (arrow).

View larger version (134K): [in a new window]   Figure 13b.  (a) Sagittal T1-weighted MR image demonstrates posterior and superior elevation of the right cavernous internal carotid artery (arrows). (b) Composite MR angiogram obtained in a different patient shows posterior displacement of the right posterior cerebral artery (arrowhead) and lateral displacement of the right cavernous carotid artery (arrow). (c) Coronal T1-weighted MR image obtained in a third patient shows a tumor with extension to the right cavernous sinus and concomitant displacement and partial encasement of the right cavernous internal carotid artery (arrow).

View larger version (184K): [in a new window]   Figure 13c.  (a) Sagittal T1-weighted MR image demonstrates posterior and superior elevation of the right cavernous internal carotid artery (arrows). (b) Composite MR angiogram obtained in a different patient shows posterior displacement of the right posterior cerebral artery (arrowhead) and lateral displacement of the right cavernous carotid artery (arrow). (c) Coronal T1-weighted MR image obtained in a third patient shows a tumor with extension to the right cavernous sinus and concomitant displacement and partial encasement of the right cavernous internal carotid artery (arrow).

View larger version (151K): [in a new window]   Figure 14a.  (a) Sagittal contrast-enhanced T1-weighted MR image shows a large, irregularly enhancing mass (arrow) with retroclival extension that encases the left internal carotid artery (arrowhead). (b) Left carotid arteriogram shows narrowing of the distal left internal carotid artery (arrow) and upward displacement of the left middle cerebral artery (arrowhead).

View larger version (138K): [in a new window]   Figure 14b.  (a) Sagittal contrast-enhanced T1-weighted MR image shows a large, irregularly enhancing mass (arrow) with retroclival extension that encases the left internal carotid artery (arrowhead). (b) Left carotid arteriogram shows narrowing of the distal left internal carotid artery (arrow) and upward displacement of the left middle cerebral artery (arrowhead).

View larger version (168K): [in a new window]   Figure 15a.  (a) Sagittal T1-weighted MR image shows a mass that involves the posterior fossa (arrows) with extracranial soft-tissue extension to the face. (b) Left external carotid arteriogram demonstrates recurrent tumor with vasculature from the branches of the posterior auricular artery (arrow). Note the occlusion of the internal carotid artery (arrowhead).

View larger version (147K): [in a new window]   Figure 15b.  (a) Sagittal T1-weighted MR image shows a mass that involves the posterior fossa (arrows) with extracranial soft-tissue extension to the face. (b) Left external carotid arteriogram demonstrates recurrent tumor with vasculature from the branches of the posterior auricular artery (arrow). Note the occlusion of the internal carotid artery (arrowhead).

View larger version (157K): [in a new window]   Figure 16a.  Anterior extension. (a) Sagittal T1-weighted MR image shows a lesion that involves the sphenoid sinus (arrows). (b) Coronal T1-weighted MR image shows orbital involvement by the lesion, with greater involvement on the left side (arrow) than on the right (arrowhead).

View larger version (166K): [in a new window]   Figure 16b.  Anterior extension. (a) Sagittal T1-weighted MR image shows a lesion that involves the sphenoid sinus (arrows). (b) Coronal T1-weighted MR image shows orbital involvement by the lesion, with greater involvement on the left side (arrow) than on the right (arrowhead).

View larger version (142K): [in a new window]   Figure 17a.  Lateral extension. (a) Coronal contrast-enhanced T1-weighted MR image demonstrates bilateral involvement of the cavernous sinuses (arrows), with more involvement on the left side than on the right. (b) Axial contrast-enhanced T1-weighted MR image demonstrates involvement of the middle cranial fossa (arrows).

View larger version (175K): [in a new window]   Figure 17b.  Lateral extension. (a) Coronal contrast-enhanced T1-weighted MR image demonstrates bilateral involvement of the cavernous sinuses (arrows), with more involvement on the left side than on the right. (b) Axial contrast-enhanced T1-weighted MR image demonstrates involvement of the middle cranial fossa (arrows).

View larger version (174K): [in a new window]   Figure 18.  Posterior extension. Axial T1-weighted MR image shows posterior fossa involvement (arrows).

View larger version (184K): [in a new window]   Figure 19.  Inferior extension. Sagittal T1-weighted MR image shows nasopharyngeal involvement (arrow).

View larger version (134K): [in a new window]   Figure 20.  Superior extension. Coronal contrast-enhanced T1-weighted MR image shows a tumor with extension into the third ventricle (arrow).

View larger version (181K): [in a new window]   Figure 21a.  (a) Presurgical axial contrast-enhanced T1-weighted MR image shows a tumor that involves the posterior fossa (arrow). (b) Axial contrast-enhanced T1-weighted MR image obtained after surgery (cranio-orbitozygomatic approach) shows complete resection of the tumor (arrows).

View larger version (146K): [in a new window]   Figure 21b.  (a) Presurgical axial contrast-enhanced T1-weighted MR image shows a tumor that involves the posterior fossa (arrow). (b) Axial contrast-enhanced T1-weighted MR image obtained after surgery (cranio-orbitozygomatic approach) shows complete resection of the tumor (arrows).

View larger version (188K): [in a new window]   Figure 22a.  (a) Preoperative sagittal T1-weighted MR image demonstrates a tumor with clival and intradural extension (arrows). (b) Sagittal T1-weighted MR image obtained after surgery performed through the midline (transmaxillary approach) reveals complete resection of the tumor (arrow).

View larger version (170K): [in a new window]   Figure 22b.  (a) Preoperative sagittal T1-weighted MR image demonstrates a tumor with clival and intradural extension (arrows). (b) Sagittal T1-weighted MR image obtained after surgery performed through the midline (transmaxillary approach) reveals complete resection of the tumor (arrow).

View larger version (149K): [in a new window]   Figure 23a.  (a) Presurgical axial CT scan shows a mass primarily in the sellar area (arrow). A transmaxillary surgical approach was used to resect the tumor. (b) Axial contrast-enhanced T1-weighted MR image obtained 1 year after surgery shows recurrent tumor at the surgical site in the nasal region (arrows). (c) Postsurgical sagittal CT scan shows recurrent tumor in the clival area (arrow).

View larger version (160K): [in a new window]   Figure 23b.  (a) Presurgical axial CT scan shows a mass primarily in the sellar area (arrow). A transmaxillary surgical approach was used to resect the tumor. (b) Axial contrast-enhanced T1-weighted MR image obtained 1 year after surgery shows recurrent tumor at the surgical site in the nasal region (arrows). (c) Postsurgical sagittal CT scan shows recurrent tumor in the clival area (arrow).

View larger version (80K): [in a new window]   Figure 23c.  (a) Presurgical axial CT scan shows a mass primarily in the sellar area (arrow). A transmaxillary surgical approach was used to resect the tumor. (b) Axial contrast-enhanced T1-weighted MR image obtained 1 year after surgery shows recurrent tumor at the surgical site in the nasal region (arrows). (c) Postsurgical sagittal CT scan shows recurrent tumor in the clival area (arrow).

View larger version (123K): [in a new window]   Figure 24a.  (a) Postsurgical axial CT scan shows a soft-tissue mass in the left infratemporal fossa (arrow). (b) Axial CT scan of the thoracic spine shows a destructive metastatic lesion in the left pedicle with adjacent soft-tissue extension (arrows). This finding represents distant metastatic spread of chordoma.

View larger version (148K): [in a new window]   Figure 24b.  (a) Postsurgical axial CT scan shows a soft-tissue mass in the left infratemporal fossa (arrow). (b) Axial CT scan of the thoracic spine shows a destructive metastatic lesion in the left pedicle with adjacent soft-tissue extension (arrows). This finding represents distant metastatic spread of chordoma.