Download Carotid Paraganglioma

Brian S. Shah HMS III
Gillian Lieberman, MD
May 2003
Carotid Paraganglioma
Brian S. Shah, HMS III
Gillian Lieberman, MD
Brian S. Shah HMS III
Gillian Lieberman, MD
BACKGROUND
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Paraganglion tissue 1st descibed by Von Haller in 1743
Tissue contains catecholamine + tryptophan granules
Histologically and functionally = adrenal medulla
Located at:
– carotid, aorticopulmonary, para-aortic and coccygeal bodies
– urinary bladder, gall bladder, heart
• 3 tissue components
– 1. Type I/chief cells (catecholamine granules)
– 2. Type II/sustentacular cells
– 3. Capillary network
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Brian S. Shah HMS III
Gillian Lieberman, MD
BACKGROUND
• Tumors of this tissue are called paragangliomas
• Nomenclature has been confusing!
– glomus tumors, chemodectomas, non chromaffin
tumors, chromaffin tumors…
– based on staining, function and adjacent structures
• Glenner and Grimely in 1974 standardized naming
based on adrenal vs. extra-adrenal sites
– now there are pheochromocytomas of the adrenal and
paragangliomas, further specified by site
• note: many authors still use the original terminology
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Brian S. Shah HMS III
Gillian Lieberman, MD
BACKGROUND
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10% multicentric
7-9% familial
2-15% malignant
• 90% are adrenal
pheochromocytomas
• 10% extra-adrenal:
– 85% in abdomen
– 12% in thorax
– 3% in head and neck
1. Carotid body
2. Jugular foramen
3. Middle ear cavity
4. Course of vagus nerve
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Brian S. Shah HMS III
Gillian Lieberman, MD
BACKGROUND
• Carotid body described by Von Haller in 1743
– Medial to carotid bifurcation (5x3x1.5mm)
– Functions as baroreceptor, 02, pH receptors
• Carotid paragangliomas soft, non-tender, slowly
enlarging neck mass
– possible dysphagia, hoarseness, tongue parasthesia
– 40-60 y/o
Male=Female
– Saldana et al noted increased incidence with COPD
patients and people living at high
altitudes…chemoreceptor hyperplasia
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Brian S. Shah HMS III
Gillian Lieberman, MD
PATIENT
HPI: The patient is a 49 y/o man presenting with complaint of
non-painful, left sided, neck swelling of approximately 5
years. He believes it is increasing in size. No other
symptoms.
PMH: heroin abuse with CVA secondary to OD
MEDS: ASA 325mg PO qd
Serax PO qhs
NKDA
PE: revealed soft, large, left sided neck mass with no other
focal findings
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Brian S. Shah HMS III
Gillian Lieberman, MD
CT IMAGING OF PARAGANGLIOMAS
• 2.5-3.O mm axial slices from thoracic inlet to skull base
• Excellent for imaging bony detail
• Win et. al. describes “… a well marginated ovoid
mass…which splays the internal and external carotid
arteries at the level of the bifurcation and demonstrates
intense homogenous enhancement following the
intravenous administration of iodinated contrast.”
• May appear similar to schwannoma but can differ using
dynamic bolus CT or MRI
• Must consider ionizing radiation and contrast
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Brian S. Shah HMS III
Gillian Lieberman, MD
CT WITH CONTRAST
MANDIBLE
MYLOHYOID
MUSCLE
EXTERNAL CAROTID A.
5cm MASS
INTERNAL CAROTID A.
EXTERNAL
JUGULAR V.
INTERNAL JUGULAR V.
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Axial CT w/ contrast courtesy of Mike Stella, MD BIDMC
Brian S. Shah HMS III
Gillian Lieberman, MD
CT FINDINGS
Axial CT + contrast
Axial CT + contrast
Axial CT bone window
Lustrin ES, Palestro C, Kirubara V: Radiographic Evaluation and Assessment of
Paragangliomas. Otolaryngologic Clinics of North America 34(5) Oct 2001
http://brighamrad.harvard.edu/education/online/tcd/tcd.html
CAROTID PARAGANGLIOMA
METASTASIS TO BONE
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Brian S. Shah HMS III
Gillian Lieberman, MD
3D CT RECONSTRUCTION
Lustrin ES, Palestro C, Kirubara V: Radiographic Evaluation and Assessment of Paragangliomas.
Otolaryngologic Clinics of North America 34(5) Oct 2001
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Brian S. Shah HMS III
Gillian Lieberman, MD
MRI IMAGING OF PARAGANGLIOMAS
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Allows imaging of lesion, surrounding nerves and vessels without
ionizing radiation
Allows mutiplanar imaging without repositioning patient
Not as good as CT at imaging bone and ear structures
T1 imaging shows a lesion intensity =or> than muscle and > than
muscle on T2 and following gadolinium
Punctate, serpentine or channel-like, hypointense flow voids should be
noted… creating a “salt and pepper appearance”
Mass effect is noted
Time-of-flight magnetized bolus of blood allows clear imaging of
vessels for brief periods of time
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Brian S. Shah HMS III
Gillian Lieberman, MD
MRI
T1 inversion axial MRI courtesy of Mike Stella, MD BIDMC
Time of flight MRI courtesy of Mike Stella, MD BIDMC
SPLAYING OF EXTERNAL AND INTERNAL CAROTID A.A.
TRACHEAL DEVIATION TO THE RIGHT
“SALT AND PEPPER” TUMOR APPEARANCE
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Brian S. Shah HMS III
Gillian Lieberman, MD
CORONAL T1 MRI
NOTE: TUMOR IS
HYPERINTENSE
COMPARED TO
MUSCLE
TUMOR
CAROTID BIFURCATION
COMMON CAROTID A.
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T1 inversion coronal MRI courtesy of Mike Stella, MD BIDMC
Brian S. Shah HMS III
Gillian Lieberman, MD
T2 WEIGHTED MRI SHOWING HYPERINTENSE
LESIONS
http://brighamrad.harvard.edu/education/online/tcd/tcd.html
Lustrin ES, Palestro C, Kirubara V: Radiographic Evaluation and Assessment of
Paragangliomas. Otolaryngologic Clinics of North America 34(5) Oct 2001
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Brian S. Shah HMS III
Gillian Lieberman, MD
MRA vs. CONVENTIONAL
ANGIOGRAPHY
• In MRA no contrast is given. Magnetized blood can be
visualized only temporarily. Only rapidly filling vessels
will be imaged and many tumor”feeders” will not be seen.
Thus the lack of tumor “blush” is normal on MRA. Large
vessels will be imaged showing mass effect.
• Conventional angiography will show the tumor “blush”
and is important if embolization is to be attempted pre-op
to minimize intra-op bleeding.
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Brian S. Shah HMS III
Gillian Lieberman, MD
NORMAL ANATOMY
INTERNAL CAROTID A.
EXTERNAL CAROTID A.
VERTEBRAL A.
CAROTID
BIFURCATION
MRA courtesy of Mike Stella, MD BIDMC
http://www.bartleby.com/107/
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Brian S. Shah HMS III
Gillian Lieberman, MD
2D MRA
NORMAL
RT
ABNORMAL
ABNORMAL
LT
LT
SPLAYING OF INTERNAL AND EXTERNAL
CAROTID A.A.
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MRA courtesy of Mike Stella, MD BIDMC
Brian S. Shah HMS III
Gillian Lieberman, MD
3D MRA
NORMAL
RT
ABNORMAL
LT
ABNORMAL
LT
SPLAYING OF INTERNAL AND
EXTERNAL CAROTID A.A.
3D MRA courtesy of Mike Stella, MD BIDMC
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Brian S. Shah HMS III
Gillian Lieberman, MD
ANGIOGRAM
ECA
ICA
LVA
Left vertebral artery angiogram courtesy of Mike Stella, MD BIDMC
LCCA
Left common carotid artery angiogram courtesy of Mike Stella, MD BIDMC
NOTE “TUMOR BLUSH” AND SPLAYING OF INTERNAL AND EXTERNAL CAROTID A.A.
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Brian S. Shah HMS III
Gillian Lieberman, MD
UTRASOUND
• Gray scale ultrasound is used to delineate tumor
size, margins and location
• Typically a well-defined, hypoechoic
heterogeneous, mass is noted splaying the ICA
and ECA
• Hypervascularity is noted on color doppler
utrasound
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Brian S. Shah HMS III
Gillian Lieberman, MD
GRAY SCALE ULTRASOUND
HYPOECHOIC
WELL
DEFINED
MASS
Left sagittal ultrasound courtesy of Mike Stella, MD BIDMC
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Brian S. Shah HMS III
Gillian Lieberman, MD
COLOR DOPPLER ULTRASOUND
HYPERVASCULAR
MASS SPLAYING
INTERNAL AND
EXTERNAL
CAROTID A.A.
Left transverse color doppler ultrasound courtesy of Mike Stella, MD BIDMC
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Brian S. Shah HMS III
Gillian Lieberman, MD
RADIONUCLIDE IMAGING
• Octreotide is labeled with 111 Indium-labeled-DTPA
(pentetreotide)
• Bind somatostatin type 2 receptors common to
paragangliomas
• advocated if suspect multicentricity in familial disease or
to image postoperatively
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Brian S. Shah HMS III
Gillian Lieberman, MD
PENTETREOTIDE SCINTIGRAPHY
33 y/o with familial h/o
paragangliomas. Presented with B
neck masses…B paragangliomas
noted
74 y/o with hoarseness thought to be hemangioma…showing
L neck paraganglioma
Lustrin ES, Palestro C, Kirubara V: Radiographic Evaluation and Assessment of
Paragangliomas. Otolaryngologic Clinics of North America 34(5) Oct 2001
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Brian S. Shah HMS III
Gillian Lieberman, MD
DIFFERENTIAL DIAGNOSIS CAROTID SPACE MASS
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Inflammatory
– abscess
Pseudotumor
– carotid artery ectasia
Vascular
– ICA dissection
– carotid aneurysm/thrombosis
– jugular vein thrombosis
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Benign tumor
– carotid body, jugular,vagal
paraganglioma
– schwannoma
Malignant tumor
– squamous cell carcinoma
– NHL
Abscesses appear as homgenous fluid filled lesions/no hypervascularity
Ectasia would bee seen on MRA and angiography
All the vascular lesions would be elucidated on MRA and angiography
Malignancy is always possible…here there is a circumscribed, non-invasive appearance coupled with slow growth on Hx
Schwannomas have a hyperintense appearance on CT but do not exhibit “salt and pepper” appearance on MRI
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Paraganglioma is most likely given MRI appearance and vascular “blush”…the lesion is isolated to the carotid bifurcation
Brian S. Shah HMS III
Gillian Lieberman, MD
TREATMENT
• External embolization of the tumor
preoperatively with 2mm microcoils
• Excision of left carotid body tumor with
interposition nonreverse vein graft from
common carotid to the internal carotid
artery
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Brian S. Shah HMS III
Gillian Lieberman, MD
REFERENCES
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Jeroen C. Jansen, MD, Robert J. Baatenburg de Jong, MD, PhD, Jaap Schipper, MD, Phd,
Andel G. L. van der mey, MD PhD, Adrian P.G. van Gils, MD, PhD. Color Doppler Imaging of
Paragangliomas in the Neck. Journal of Clinical Ultrasound 1997; 25(9): 481-485.
Lustrin E, Palestro C, Vaheesan K. Radiographic Evaluation and Assessment of
Paragangliomas. Otolaryngologic Clinics of North America 2001; 34(5)
McCaffrey T, Myssiorek D, Marrinan M. Head and Neck Paragangliomas Physiology and
Biochemistry. Otolaryngologic Clinics of North America 2001; 34(5)
Myssiorek D. Head and Neck Paragangliomas an Overview. Otolaryngologic Clinics of North
America 2001; 34(5)
Olsen W, Dillon W, Kelly W, Norman D, Brant-Zawadzki M, Newton T. MR Imaging of
Paragangliomas. AJR 1987; 148:201-204.
Wasserman P, Savargaonkar P. Paragangliomas Classification, Pathology, and Differential
diagnosis. Otolaryngologic Clinics of North America 2001; 34(5)
Win T, Lewin J. Imaging Characteristics of Carotid Body Tumors. American Journal of
Otolaryngology 1995; 16(5):325-328
http://brighamrad.harvard.edu/education/online/tcd/tcd.html
http://www.bartleby.com/107/
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Brian S. Shah HMS III
Gillian Lieberman, MD
ACKNOWLEDGEMENTS
Special thanks to:
• Mike Stella, MD
• Larry Barbaras and Cara Lyn D’amour
our Webmasters
• Gillian Lieberman, MD
• Pamela Lepkowski
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