Download The Clivus Lesions: Crossroads Of Skull Base

The Clivus Lesions: Crossroads Of Skull Base
Poster No.:
C-1617
Congress:
ECR 2014
Type:
Educational Exhibit
Authors:
E. S. Morales Deza, E. Santamarta, A. Saiz Ayala, E. Murias, P.
Vega Valdes, J. L. LLorente Pendas; Oviedo/ES
Keywords:
Head and neck, Musculoskeletal bone, MR, CT, Imaging
sequences, Education and training
DOI:
10.1594/ecr2014/C-1617
Any information contained in this pdf file is automatically generated from digital material
submitted to EPOS by third parties in the form of scientific presentations. References
to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in
any way constitute or imply ECR's endorsement, sponsorship or recommendation of the
third party, information, product or service. ECR is not responsible for the content of
these pages and does not make any representations regarding the content or accuracy
of material in this file.
As per copyright regulations, any unauthorised use of the material or parts thereof as
well as commercial reproduction or multiple distribution by any traditional or electronically
based reproduction/publication method ist strictly prohibited.
You agree to defend, indemnify, and hold ECR harmless from and against any and all
claims, damages, costs, and expenses, including attorneys' fees, arising from or related
to your use of these pages.
Please note: Links to movies, ppt slideshows and any other multimedia files are not
available in the pdf version of presentations.
www.myESR.org
Page 1 of 53
Learning objectives
The aim of this study is to show the radiological findings of the common and uncommon
clival lesions including advanced MR sequences.
Background
The clivus is a "hidden" part of the base of the skull located between the foramen
magnum and back of the sella and so far difficult to explore. Radiologists play a key
role in the demonstration and description of the clivus pathology. There are many
disease processes involving clivus, that are mixed due to their histological origin (bone,
cartilage, notochord residues) and highly vascularized nature (metastasis). There are
also "pseudo-lesions" or "incidentalomas" that we should to know and distinguish from
the significant injuries. Endoscopic treatment techniques are becoming more common,
which has increased the interest in this area.
Anatomy:
The clivus is a bony canal in the basilar of occipital bone, between the back of the sella
and the foramen magnum. It is formed by the union of the posteroinferior part of the body
of the sphenoid bone (basisphenoid) and the anterosuperior part of the occipital bone
(basioccipital).
The sphenoid synchondrosis is the junction between the sphenoid and occipital bones,
which usually ends between 18-25 years.
Anatomical relations:
•
•
•
•
Anterior: Sphenoidal sinus.
Posterior: Cisterns of the skull base.
Inferior: Nasopharynx. Atlanto-occipital joint.
Lateral: Petro-occipital fissures and condylar canals.
The signal of cancellous bone depends on the degree of maturity of the bone marrow. The
hematopoietic tissue, which predominates in young individuals, is usually hypointense
on T1WI MR, but when fat metamorphosis occurs, it becomes more hyperintense.
Most of lesions of the clivus are hypointense on T1WI and hyperintense on T2WI MR.
The imaging techniques to study the skull base commonly include the MDCT and MRI,
the last one with conventional (T1,T2, FST2, GRT2*, FLAIR, Dynamic GRE +C, T1 FS +
Page 2 of 53
C), and special sequences (DWI, 3D FIESTA, etc). Each of them has its own contributions
to the diagnosis.
Images for this section:
Fig. 1: Endoscopic treatment techniques are becoming more common, which has
increased the interest in this area.
Page 3 of 53
Fig. 2
Page 4 of 53
Fig. 3
Page 5 of 53
Fig. 4
Page 6 of 53
Fig. 5
Page 7 of 53
Fig. 6: Pneumatization begins at birth, grows backwards to invade the basisphenoid (BS)
Page 8 of 53
Fig. 7: Bone marrow is composed predominantly of fat and water, Relative proportions
of these, affect the signal intensity in MRI.
Page 9 of 53
Fig. 8
Page 10 of 53
Fig. 9: CT identifies lytic or blastic lesions, and the presence of cartilage matrix or
calcifications with great sensitivity. It is less sensitive to the infiltration of the bone marrow.
Page 11 of 53
Fig. 10: The natural contrast of fat in adult bone marrow let to identify focal or infiltrative
lesions with high sensitivity.
Page 12 of 53
Fig. 11: Metastasis of Prostate: Small focal sclerotic lesion on CT and hypointense on
T1WI, that increases its size in one year.
Page 13 of 53
Fig. 12: Infiltrative tumor lesions alter the sign of the recognizable bone marrow in
diffusion sequence (b 1000) as hyper intense areas.
Page 14 of 53
Fig. 13: The detection of small "residual tumor" or "abscess" also benefits from the
diffusion sequence.
Page 15 of 53
Fig. 14: The presence of intramedullary enhancement in the clivus helps to detect
metastatic lesions in doubtful cases.
Page 16 of 53
Fig. 15: Presence of intra medullary enhancement helps to differentiate the bone edema
(because of tumor proximity) from the true tumor infiltration.
Page 17 of 53
Findings and procedure details
This is a retrospective study of 62 patients with lesions involving the clivus between
october 2006 and july 2013 in our database (HUCA). All patients were studied with
a equipment of MRI 1.5 Tesla GE Signa MR LX and two multi-slice MDCT Toshiba
scenners 16/64 channels.
RESULTS:
1.-Primary Neoplasms: 11
Chordoma (8), chondrosarcoma (2), enchondroma (1)
2.-Secondary tumors: 34
Metastasis (16), nasopharyngeal carcinoma (7), rhabdomyosarcoma (1),
juvenile angiofibroma (2), invasive pituitary adenoma (3) and
meningiomas(5)
3.-Hematopoietic Disorders: 8
Myeloma (4), leukemias (2), histiocytosis (2)
4.-Pseudotumoral or dysplastic lesions: 11
Arachnoid granulations (2), intraosseous lipomas (3), fibrous dysplasia
(2), Paget (1), simple cyst (1), meningocele (1), cholesterol cyst (1).
5.-Inflammatory and infectious lesions: 4
Osteomyelitis (1), mucopyocele (2), radiotherapy (1)
6.-Miscelanea: 3
Fístula (1), fracturas (2).
Because his mixed histological origin (bone, chordomatous, cartilage, notochordal rest..)
and his highly vascularised nature the are many pathological processes involving clivus
which represent a diagnostic and a therapeutic challenge. MDCT appears to be a perfect
diagnostic tool when bony involvement is present. So, TC is useful when cortical bone
is affected or if obvious lytic or sclerotic bone lesions are present. Fibro-osseous and
Page 18 of 53
calcified lesions are also well seen on MDCT. MRI appears to be more sensitive in
detecting bone marrow involvement in the clivus and occipital bone. Isolated bone
marrow metastasis or diffuse infiltrative processes of the skull base is best demonstrated
on MRI. Sagittal and axial SE-T1 weighted and Dynamic GRE Gadolinium enhanced
T1-weighted MR images are the most useful sequences. T2-weighted images are very
sensitive for chordomas and chondrosarcomas. The apparent diffusion coefficient (ADC)
value, determined from DWI, can help in cancer staging and detection small clivus
metastasis or postoperative residual tumor. The ADC value also discriminates tumors
from abscesses.
Images for this section:
Fig. 16
Page 19 of 53
Fig. 21: Rare malignant neoplasm (1% of intracranial T.), slow-growing, derived from
remnants of the notochord. Affects the midline. 35% at the skull base: clivus: more
frequent (spheno-occipital synchondrosis), sphenoid (30%) and petrous apex.
Page 20 of 53
Fig. 18
Page 21 of 53
Fig. 17: a) Sag SE T1WI + C: large sphenoidal mass extending from the Sellar area to the
rhino pharynx. b) Sag CT + C: erosion of the upper third of the clivus. c) Coronal T2-FSE:
heterogeneous hyperintense mass in nasopharynx. d) Axial SE T1WI + C and FS : The
lesion shows homogeneous enhancement and erosion of the lateral portion of the clivus.
Page 22 of 53
Fig. 22: Slow growing chondroid neoplasm. They may occur in cartilage, endochondral
bone or primitive mesenchymal cells of the brain, meninges or membranous bone of soft
tissue. It may be secondary to bone diseases (fibrous dysplasia, Paget's disease, Ollier
syndrome).
Page 23 of 53
Fig. 19: Petroclival chondrosarcoma A) and B) Axial CT: typical chondroid calcifications.
C)GR T2 * intratumoral calcifications. D)Axial SE T1 + C: parasellar lesion with little
enhancement (heterogeneous) with clival involvement which extends to prepontine
cistern. E) FSE T2 Fat Sat. F) DWI MR: Deformity of the lesion by magnetic susceptibility
of the skull base and air interface.
Page 24 of 53
Fig. 20: A) and B) Axial CT: petroclival lytic destructive lesion in the petro-occipital
fissure. Small foci of calcifications with tumoral matrix are seen. C) Sag T1WI: clival mass
with medial extension to prepontine cistern that compresses the brainstem. D) Diagram
of skull base. E) and F) Axial and coronal SE T2 FS: hyperintense tumor with small
hypointense foci.
Page 25 of 53
Fig. 23: It particularly affects patients with breast carcinoma, lung, kidney and prostate.
There are 5 syndromes according to metastasis location (orbitary, parasellar, middle
cranial fossa, jugular foramen and condylar)
Page 26 of 53
Fig. 24
Page 27 of 53
Fig. 25: Metastasis. In adults T1WI sequences are more sensitive than CT or T2.
Diffusion sequences are helpful in selected cases, and dynamic sequences with contrast
show in a better way the abnormal medullary enhancement. We report 2 cases with
doubtful CT where DWI and dynamic study established the diagnosis.
Page 28 of 53
Fig. 26: Highly invasive tumor, up to 30% with invasion of the skull base until initial
diagnosis (because of paucity of symptoms).
Page 29 of 53
Fig. 27: Bone invasion. A) and B) Axial SE without and with contrast: Infiltrative enhancing
mass that originates from the nasopharynx, with bone destruction of the skull base,
affecting the clivus, foramen ovale and foramen lace rum. C) Coronal SE FS + C: It
infiltrates the left cavernous sinus and surrounds the ipsilateral carotid artery. D) Axial T2
FSE: shows an infiltrative pattern in the clivus and petrous apex.
Page 30 of 53
Fig. 28: Sinus tumors are 2-3% of the head and neck tumors. Only 1-2% affect the
sphenoid sinus. Histology: Squamous carcinoma (70-80%), only 6-17% are tumors from
minor salivary glands, mucoepidermoid and undifferentiated carcinomas. A) Sagittal SET1: Expasive tumor (T) of the sphenoid sinus which extends to the nose and clivus.
B) Coronal T2 FSE: sphenoid sinus expanded laterally and compression of the left
cavernous sinus.
Page 31 of 53
Fig. 29: 5% of pituitary adenomas can become "invasive" and simulate metastases or
other aggressive tumors. A invasive adenoma, but benign, could not be distinguished
from a pituitary carcinoma on imaging.
Page 32 of 53
Fig. 30: A) and B) Axial CT: large expansive lytic lesion in the right sellar and parasellar
region with typical benign bone margins. C) and D) Cor and Axial T1 + C: sellar mass
that invades the skull base and extends through the right cavernous sinus and clivus. E)
Sag T1 + C: cranially suprasellar extension surrounding the carotid. F) Diagram of the
skull base.
Page 33 of 53
Fig. 31: Benign tumors that arise from arachnoid cells of the meninges (15% of all primary
brain tumors). Any sphenoid meningioma can extend extracranially through foramina of
the skull base or by destruction and direct extension from the middle cranial fossa.
Page 34 of 53
Fig. 32
Page 35 of 53
Fig. 33: MM represents >40% of primary malignant bone tumors. It is the most common
primary malignant bone tumor.
Page 36 of 53
Fig. 34: Plasmacytoma of skull base: A) Axial CT: diffuse destructive/lytic areas in the
center of the base of the skull with sellar infiltration, basisphenoid and ethmoid. B)
Diagram of the skull base. C) and D) Axial CT + C: Infiltrating soft tissue mass with
moderate enhancement that affects the central skull base and both petrous apices.
Multiple calcifications.
Page 37 of 53
Fig. 35: Non-Hodgkin lymphoma (NHL) is a rare tumor of the skull base. Nasal and
paranasal sinus NHL, with involvement of the skull base and intracranial affectation,
represents advanced sinonasal primary disease.
Page 38 of 53
Fig. 36: Diffuse infiltration of the bone marrow in hematological tumor processes is
nonspecific and comparable to metastatic infiltration.
Page 39 of 53
Fig. 37: Mucopyocele by Staphylocus hyicus (cocaine abuse): A), B) and C) Sag FSE
T2 and T1 SE with and without contrast: expansive lesion in quadrilateral plate and
clivus with cortical destruction. Occupation of the frontal sinus. Peripheral enhancement
of the mucosa with central hypointensity in both lesions. D) and E) Axial DWI: signal
hyperintensity with restrictrion on diffusion, indicative of abscesses. F) and G) axial and
cor CT: bone destruction of clivus.
Page 40 of 53
Fig. 38: Malignant otitis externa by Pseudomona aeruginosa in diabetic patient: A) and
B) CT shows sclerosis in the right side of the clivus consistent with osteomyelitis by
contiguity. C) and D) T1WI MR shows a large soft tissue inflammatory component of the
masticator and parapharyngeal spaces with bone infiltration, compatible with edema and/
or osteomyelitis.
Page 41 of 53
Fig. 39: Lesión pseudotumoral de morfología redonda, ovoidea y densidad similar a LCR
en todas las secuencias estables en el tiempo, Normalmente son de pequeño tamaño,
aunque existen forma pseusotumorales y en ocasiones se asocian a fístulas de LCR. A)
sagital en T1, B) coronal en T2 y C) axial de TC con ventana ósea: lesión hipointensa
en T1 e hiperintensa en T2, de bordes bien definidos y esclerosos.
Page 42 of 53
Fig. 40: It is usually asymptomatic and incidentally discovered in CT or MRI. In TC can
simulate a more aggressive lesion such as chroma. MRI fat suppression techniques
allows diagnosis. A) CT axial, B) sagital T1WI, C) axial T1WI, D) FLAIR, E) T2WI, and F)
Fat-suppressed T1: bone lesions with thin hypointense edges in the body of the sphenoid
(secondary to reactive sclerosis), and hyperintense centre on T1 and T2WI wihch is
suppressed on FS sequences.
Page 43 of 53
Fig. 41: Cholesterol granuloma of the petrous apex: It is the most common lesion on the
petrous apex, ad produces a characteristic expansive lytic lesion with cortical thinning,
secondary to repeated bleeding. Petro-clival expansive lytic lesion. Sag SE T1WI shows
the characteristic signal intensity due to the presence of chronic hematic elements and
crystals of cholesterol (xanthochromic liquid). SE-T1 Fat Sat: to differentiate it from other
lipomas. SE-T1 +C : no enhancement. DWI MR: heterogeneous hypointense signal (T2
dark through) by the presence of hemosiderin.
Page 44 of 53
Fig. 42: Osteochondromas are uncommon in the region of the clivus. They can cause
local mass effect and rarely compromise of the foramen magnum. Usually asymptomatic.
A) 3D CT axial reconstrucción, B) and C) MPR CT coronal and sagital, D) and E) axial
FLAIR and sagittal T1 SE MRI: hyperostotic bone reaction of the lower-left region of the
clivus, and "pseudoarticulation" with the odontoid.
Page 45 of 53
Fig. 43: Defects of neural tube closure during early embryonic development, with
"herniation" of the dura through a bone defect, containing meninges and CSF. Signal
equal to CSF on all MRI sequences (T1, T2, fat suppression). CT scan shows well-defined
erosions of the clivus and petrous apex, with water density.
Page 46 of 53
Fig. 44: Sagittal, axial and coronal CT with bone windowing: lytic and sclerotic multiple
lesions distributed throughout the calotte and the skull base, which also affect the clivus.
Page 47 of 53
Fig. 45: Also called Osteitis deformans: CT: varies according to the phase of the disease:
Destructive osteoporotic/lytic or osteoclastic phase with forming bone. Simple RX and
CT: Extensive bone involvement with thickening and "cotton wool" appearance in all the
calotte and skull base. A) and B) CT in advanced stage: widening and thickening of the
entire skull base and calotte, with "cotton wool" densities. Deformity and bone remodeling
are characteristic.
Page 48 of 53
Fig. 46: Axial and 3D reconstruction CT: expansive bone lesion with variable attenuation.
Lesion with lucente centre, thin sclerotic margins, and "ground glass" density. Sphenoethmoidal involvement of the clivus and thickening due to fibrous dysplasia.
Page 49 of 53
Fig. 47: CT (A, B, C and D), 3D CT (E): fracture lines, one horizontal across the upper
third of the clivus (#) with avulsion of the sellar dorsum (#), sinus hemorrhage (* ) and
pneumocephalus in a patient with severe TBI.
Page 50 of 53
Fig. 48: A) and B) Sagittal T2 and T1WI MR: Pneumatization with fluid level in the
sphenoid sinus (*). C) and D) axial and sagittal Mielo CT: bone dehiscence in the upper
third of the clivus (basisphenoid) (#). E) Sagittal T2WI MR: hypointense postoperative
material (tensor Fascia Latae) plugging the fistula (#).
Page 51 of 53
Conclusion
Combination of MDCT and MRI findings allow the correct diagnostic approach to the
clivus pathology.
Bone involvement is best demonstrated on MDCT with bone windowing, while MRI
is more accurate in determining tumor margins, soft tissue involvement and diffuse
intraosseous lesions.
In our experience the most useful sequences were: T1 SE with and without Fat Sat,
dynamic sequences with contrast, and difusion.
Knowledge of tumor and tumor-like lesions of the petroclival area is important for a correct
diagnosis.
Personal information
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
Gupta A, Harnsberger HR. Chordoma, clivus. In: Diagnostic Imaging: Head
and Neck. Salt Lake City: Amirsys;2004 :I3-10
Chua R, Shapiro S. A mucopyocele of the clivus: case report. Neurosurgery
1996;39:589-90.
Oruckaptan HH, Akdemir P, Ozgen T. Isolated sphenoid sinus abscess:
clinical and radiological failure in preoperative diagnosis. Case report and
review of the literature. Surg Neurol 2000;53:174-77.
Nemeth AJ, Henson JW, Mullins ME, et al. Improved detection of skull
metastasis with diffusion-weighted MR imaging. AJNR Am J Neuroradiol
2007;28:1088-92.
Erdem E, Angtuaco EC, Van Hemert R, et al. Comprehensive review of
intracranial chordoma. Radiographics 2003;23:995-1009.
Hayashida Y, Hirai T, Yakushiji T, et al. Evaluation of diffusion-weighted
imaging for the differential diagnosis of poorly contrast-enhanced and
T2-prolonged bone masses: initial experience. J Magn Reson Imaging
2006;23:377-82 .
Pui MH, Mitha A, Rae WI. Diffusion-weighted magnetic resonance imaging
of spinal infection and malignancy. J Neuroimaging 2005;15:164-70 .
Kainberger F. Diffusion weighted imaging of bone marrow pathologies. Eur J
Radiol 2005;55:74-83.
Wolfe JT III, Scheithauer BW. "Intradural chordoma" or "giant ecchordosis
physaliphora"? Report of two cases. Clin Neuropath 1987;6:98-103
Page 52 of 53
10. Erdem E, Angtuaco E, Hemert R, et al. Comprehensive review of intracranial
chordoma. Radiographics 2003;23:995-1009.
11. DiFrancesco LM, Castillo C, Temple WJ. Extra-axial chordoma. Arch Pathol
Lab Med 2006;130:1871-74.
12. Masui K, Kawai S, Yonezawa T, et al. Intradural retroclival chordoma without
bone involvement. Neurol Med Chir (Tokyo) 2006;46:552-55.
13. Madeline LA, Elster AD. Postnatal development of the central skull base:
normal variants. Radiology 1995;196:757-63.
14. Hughes ML, Carty AT, White FE. Persistent hypophyseal (craniopharyngeal)
canal. Br J Radiol 1999;72:204-06.
15. Marsot-Dupuch K, Smoker WR, Grauer W. A rare expression of neural crest
disorders: an intrasphenoidal development of the anterior pituitary gland.
AJNR Am J Neuroradiol 2004;25:285-88.
16. Barnes L. Pathology of selected tumors of the base of the skull. Skull Base
Surg 1991;1:207-16.
17. Heffelfinger MJ, Dahlin DC, MacCarty CS, et al. Chordomas and
cartilaginous tumors at the skull base. Cancer 1973;32:410-20.
18. King AD, Lei KI, Richards PS, et al. Non-Hodgkin's lymphoma of the
nasopharynx: CT and MR imaging. Clin Radiol 2003;58:621-25.
19. Weber AL, al-Arayedh S, Rashid A. Nasopharynx: clinical, pathologic and
radiologic assessment. Neuroimaging Clin N Am 2003;13:465-83.
20. Urquhart A, Berg R. Hodgkin's and non-Hodgkin's lymphoma of the head
and neck. Laryngoscope 2001;111:1565-59.
Page 53 of 53