Virginia Hsu
Gillian Lieberman, MD
July 2002
Carotid
Carotid Body
Body Tumors
Tumors
Virginia Hsu, Harvard Medical School Year IV
Gillian Lieberman, MD
1
Virginia Hsu
Gillian Lieberman, MD
Our Patient
• AD is a 48 year old male with 7-8 year
history of a left neck mass
• No previous imaging studies
• On exam:
“Firm, well-circumscribed,
round L submandibular mass. No
tenderness to palpation, no bruit.”
• CT scheduled and ENT consulted
2
Virginia Hsu
Gillian Lieberman, MD
January 2002: Axial CT w/
Contrast
Mandible
Submandi
bular g.
External
Carotid a.
Internal
Carotid a.
Internal
Jugular v.
Mass
SCM
External
Jugular v.
BIDMC PACS
Vertebral
a.
3
Virginia Hsu
Gillian Lieberman, MD
January 2002: Axial CT w/
Contrast
•Hypervascularity
demonstrated by prompt,
intense enhancement
•Located at carotid
bifurcation splaying the
internal and external
carotids
•Airway displaced to right
•No bony destruction
•No lymphadenopathy
•Well-preserved planes
BIDMC PACS
•Approximately 5cm
4
Virginia Hsu
Gillian Lieberman, MD
CT January 2002
BIDMC PACS
BIDMC PACS
Extends from
carotid bifurcation
superiorly to C1 5
Virginia Hsu
Gillian Lieberman, MD
Carotid Space Anatomy
•Internal carotid
artery
•Internal jugular
vein
•Sympathetic
chain
•Cranial nerves
IX, X, XI, XII
•Lymph nodes
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http://www.bartleby.com/107/143.html
Virginia Hsu
Gillian Lieberman, MD
DDX Carotid Space Masses
Carotid
Artery
•Ectasia
•Aneurysm
•Pseudoaneurysm
•Dissection
•Encasement by
direct spread of
SCC
Jugular Vein Cranial Nerves
•Asymmetric
enlargement
(X,XI,XII, sympathetic
chain)
•Thrombosis
•Neurogenic tumor
•Thrombophlebitis
•Neuroblastoma
•Paraganglioma
•Meningioma
(from jugular
foramen)
Lymph Nodes
Inflammatory
•Abscess
•Metastatic cervical
adenopathy
•Lymphoma
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Virginia Hsu
Gillian Lieberman, MD
Carotid
Artery
Narrowing the DDX
•Ectasia
•Aneurysm
•Pseudoaneurysm
• Mass is not
intrinsic to
blood vessels
direct spread of
SCC
•Asymmetric
enlargement
• Slow-growing, •Thrombosis
well- circum- •Thromboscribed mass
phlebitis
• No extension
from jugular
•Encasement by
foramen
•Dissection
Jugular Vein
•Meningioma
(from jugular
foramen)
Inflammatory
•Abscess
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Virginia Hsu
Gillian Lieberman, MD
Narrowing the DDX con’t
Cranial Nerves
•Hypervascularity
(X,XI,XII, sympathetic
chain)
•No calcification or necrosis
•Neurogenic tumor
•No lymphadenopathy
•Neuroblastoma
•Paraganglioma
Lymph Nodes
•Metastatic cervical
adenopathy
•Lymphoma
It must be a
paraganglioma- a
carotid body tumor!
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Virginia Hsu
Gillian Lieberman, MD
Paragangliomas
•Rare tumors that arise from specialized
neural crest cells associated with autonomic
ganglia.
•4 extradrenal locations
Group I: Great vessels of chest and neck
GroupII: Vagus nerve
Group III: Aorticosympathetic chain
Group IV: Visceral organs
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Virginia Hsu
Gillian Lieberman, MD
Paragangliomas
Head and Neck
• Carotid body
paraganglioma
• Vagal paraganglioma
(nodose ganglia)
• Glomus tympanicummiddle ear along
tympanic plexus
• Glomus jugularejugular bulb
11
Glenner, GG and Grimley PM. Tumors of the Extra-Adrenal Paraganglion System.
Bethesda, MD: Armed Forces Institute of Pathology, 1974
Virginia Hsu
Gillian Lieberman, MD
Paragangliomas
• 1/30,000 head&neck tumors are
paragangliomas
• 2-3% head/neck paragangliomas
have functional hormone secretion
• Usually benign- 6% CBTs reported
to be malignant
• Familial form (10-25%)- present
younger and with multiple tumors
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Virginia Hsu
Gillian Lieberman, MD
Carotid Body Tumors
• CB sits in adventitia at bifurcation of
common carotid a.
• Regulates respiration and maintains
arterial gases (chemoreception)
• Hyperplasia seen in chronic hypoxic
states- altitude, COPD, cyanotic heart
disease
• Presentation: avg. age= 45, slowgrowing, asymptomatic or mass-related
effects, 10% present with CN palsy
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Virginia Hsu
Gillian Lieberman, MD
Imaging Studies
•
• CT
CT
•
• MRI/MRA
MRI/MRA
•
• Ultrasound
Ultrasound
•
• Angiography
Angiography
•
• Radionuclide
Radionuclide imaging
imaging
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Virginia Hsu
Gillian Lieberman, MD
CT
• Thin section scanning from thoracic inlet
to skull base in patients with CB or vagal
paragangliomas or other palpable neck
mass
• Examines integrity of associated soft
tissues
• Detection of multiple lesions
• 3D reconstruction visualizes associated
vasculature.
15
Virginia Hsu
Gillian Lieberman, MD
CT May 2002
• No significant
interval change to
large enhancing
mass
• Displacement of
airway to right
BIDMC PACS
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Virginia Hsu
Gillian Lieberman, MD
3-D CT Reconstruction
Lustrin ES, PalestroC, Kirubahara V: Radiographic evaluation and assessment of
paragangliomas. Otolaryngologic Clinics of North America 34(5) Oct 2001
17
Virginia Hsu
Gillian Lieberman, MD
MRI
• Aids in lesion diagnosis and localization
• Differentiates mass from surrounding
inflammatory changes, fluid or vascular
structures
• More sensitive for delineating
encroachment and encasement of vessels
• Images middle ear structures and bony
erosions
• Coronal sequences
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Virginia Hsu
Gillian Lieberman, MD
T1 MRI w/o Contrast
•Paraganglioma
•Carotid
arteries
•Trachea
BIDMC PACS
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Virginia Hsu
Gillian Lieberman, MD
MRI: T2
•Well-defined mass
•heterogeneous
hyperintensity
•Punctate flow voids.
•“Salt and pepper”
BIDMC PACS
pattern: due to high
vascularity with
associated areas of
hemorrhage, slowflowing blood, and
tumor cells.
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Virginia Hsu
Gillian Lieberman, MD
MRI: T1 Coronal
• Paraganglioma
• Carotid Bifurcation
• Common Carotid
• Aortic arch
BIDMC PACS
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Virginia Hsu
Gillian Lieberman, MD
MRI: Time of Flight
• Splaying of
internal and
external carotid
arteries
• No aneurysm or
stenosis
BIDMC PACS
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Virginia Hsu
Gillian Lieberman, MD
MRA
• Noninvasive
• Delineates displacement of
vasculature
• Demonstrates tumor vascular
supply
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Virginia Hsu
Gillian Lieberman, MD
MRA-Normal
Common
Carotid a.
Internal
Carotid a.
External
Carotid a.
Vertebral
a.
http://www.bartleby.com/107/143.html
BIDMC24PACS
Virginia Hsu
Gillian Lieberman, MD
MRA
R
L
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BIDMC PACS
BIDMC PACS
Virginia Hsu
Gillian Lieberman, MD
Ultrasound
• Delineates tumor margins, size and
location
• Doppler: demonstrates hypervascularity
of paragangliomas
• Surveys neck for other lesions
• Differentiates CBTs from vascular
anomalies and pseudoaneurysms
• Can obtain US guided fine needle
aspiration
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Virginia Hsu
Gillian Lieberman, MD
Ultrasound
BIDMC PACS
Well-defined,
hypoechoic
heterogeneous
mass at carotid
bifurcation
measuring
5.7x4.2x4.1 cm
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BIDMC PACS
Virginia Hsu
Gillian Lieberman, MD
Doppler
Ultrasound
BIDMC PACS
Hypervascular
mass, splaying of
internal and
external carotid
arteries
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BIDMC PACS
Virginia Hsu
Gillian Lieberman, MD
Ultrasound-Guided Fine
Needle Aspiration
BIDMC PACS
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Virginia Hsu
Gillian Lieberman, MD
Angiography
• Demonstrates the
primary arterial
supply and collateral
vessels of tumor
• Reveals relationships
with neck blood
vessels
• Presurgery
transcatheter arterial
embolization
Lustrin ES et al: Radiographic evaluation and assessment of
paragangliomas. Otolaryngologic Clinics of N. America 34(5) Oct
2001
30
Virginia Hsu
Gillian Lieberman, MD
Radionuclide Imaging
• Pentetreotide=
octreotide
radiolabelled with 111
indium-DTPA binds
somatostatin type 2
receptors in
paragangliomas
• Uses: follow recurrent
disease, locates
multiple lesions,
detects familial
paragangliomas
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Lustrin ES et al: Radiographic evaluation and assessment of
paragangliomas. Otolaryngologic Clinics of N. America 34(5) Oct 2001
Virginia Hsu
Gillian Lieberman, MD
Metastatic CBT with Lytic
Bone Lesions
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Lustrin ES et al: Radiographic evaluation and assessment of paragangliomas. Otolaryngologic Clinics of N. America 34(5) Oct 2001
Virginia Hsu
Gillian Lieberman, MD
Familial Paraganglioma
•T1 weighted
MRI
•T2 weighted
MRI
•Angiography
Lustrin ES et al: Radiographic evaluation and assessment of
paragangliomas. Otolaryngologic Clinics of N. America 34(5)
Oct 2001
33
Virginia Hsu
Gillian Lieberman, MD
Vagal Paraganglioma
A. T1: lesion posterolateral to carotids
B. T2: Salt and pepper appearance
C. Coronal T2
D. Arteriogram: tumor blush in relation to carotids,
no splaying as in CBTs.
34
Lustrin ES et al: Radiographic evaluation and assessment of
paragangliomas. Otolaryngologic Clinics of N. America 34(5) Oct 2001
Virginia Hsu
Gillian Lieberman, MD
Glomus Jugulare
• Destructive lesion
in skull base
involving middle
ear and
hypoglossal canal
A.T1 w/o contrast
B,C. T1 w/ contrast
Lustrin ES et al: Radiographic evaluation and assessment of
paragangliomas. Otolaryngologic Clinics of N. America 34(5) Oct
2001
35
Virginia Hsu
Gillian Lieberman, MD
Glomus Tympanicum
• High resolution
axial CT of right
temporal bone
36
Lustrin ES et al: Radiographic evaluation and assessment of
paragangliomas. Otolaryngologic Clinics of N. America 34(5) Oct 2001
Virginia Hsu
Gillian Lieberman, MD
Gross Pathology
Well-defined
neoplasm with a
pseudocapsule
Glenner, GG and Grimley PM. Tumors of the Extra-Adrenal
Paraganglion System. Bethesda, MD: Armed Forces 37
Institute of
Pathology, 1974
Virginia Hsu
Gillian Lieberman, MD
Histology
Our Patient:
no malignant cells
•Small nests of bland
cells with centrally
placed hyperchromic
nuclei that form
clusters called
Zellballen (cell balls).
Glenner, GG and Grimley PM. Tumors of the Extra-Adrenal
Paraganglion System. Bethesda, MD: Armed Forces Institute of
Pathology, 1974
38
Virginia Hsu
Gillian Lieberman, MD
Our Patient…
• Followed by ENT
• Will have surgical resection of CBT
39
Virginia Hsu
Gillian Lieberman, MD
References
Glenner GG and Grimley PM. Tumors of the Extra-Adrenal Paraganglion System. Bethesda, MD: Armed
Forces Institute of Pathology, 1974
Lustrin ES et al: Radiographic evaluation and assessment of paragangliomas. Otolaryngologic Clinics
of N. America 34(5) Oct 2001
Som PM, Bergeron RT. Head and Neck Imaging. St. Louis: Mosby Year Book, 1991.
http://brighamrad.harvard.edu/Cases/bwh/hcache/74/full/html
http://www.bartleby.com/107/143.html
http://individual.uptodateonline.com/application/topic/topicText.asp?file=brain_ca/10730
Mafee MF, Raofi B, Kumar A, Muscato C. Glomus faciale, glomus jugulare, glomus tympanicum,
glomus vagale, carotid body tumors and simulating lesion. Radiologic Clinics of North America
38(5) Sept 2000
Novelline RA, Squire LF. Living Anatomy. Philadelphia: Hanley & Belfus, Inc., 1987
Myssiorek D. Head and Neck Paragangliomas: An Overview. Otolaryngologic Clinics of N. America
34(5) Oct 2001
Van der Mey AGL, Jansen JC, van Baalen, JM. Paragangliomas of the Head and Neck:Management of
carotid body tumors. Otolaryngologic Clinics of N. America 34(5) Oct 2001
Wasserman PG, Savargaonkar P. Paragangliomas of the Head and Neck: Classification, patholgy,
differential diagnosis. Otolaryngologic Clinics of N. America 34(5) Oct 2001
McCaffrey TV, Myssiorek D, Marrinana M. Paragangliomas of the Head and Neck: Physiology and
biochemistry. Otolaryngologic Clinics of N. America 34(5) Oct 2001
40
Richardson MS.Skull Base Tumor Surgery: Pathology of skull base tumors. Otolaryngologic Clinics of N.
America 34(6) Dec 2001
Virginia Hsu
Gillian Lieberman, MD
Acknowledgements
Acknowledgements
• Gwendolyn Dole, MD
• Pamela Lepkowski
• Larry Barbaras and
Cara Lyn D’Amour
• Gillian Lieberman, MD
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