Glomus Tumors of the Temporal Bone

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Glomus Tumors of the Temporal Bone
Glomus Tumors
of the Temporal Bone:
Synopsis of Glomus Tympanicum and Jugulare
Viet Pham, M.D.
Dayton Young, M.D.
The University of Texas Medical Branch (UTMB Health)
Department of Otolaryngology
Grand Rounds Presentation
May 29, 2012
Glomus Tumors
of the Temporal Bone
Synopsis of Glomus Tympanicum and Jugulare
May 29, 2012
Viet Pham
MD
(http://www.explosm.net)
All images obtained via Google search unless otherwise
specified. All images used without permission.
Dayton Young
MD
Outline





Paraganglia
Paraganglioma
Presentation
Diagnostics
Classification
Treatment
(http://me.hawkelibrary.com)
(http://www.dizziness-and-balance.com)

(http://my.clevelandclinic.org)
(Swartz 2009)
Physiology
Paraganglia

Glomus bodies

Clusters of chief cells




Identical to carotid body
Similar to adrenal autonomic ganglia
“Zellballen” network with arterioles and
venules
Chief cells


Arise from neural crest cells, migrate with
sympathetic ganglia (Gulya 1993)
Neurosecretory modulators of vascular
activity


Dopamine
Norepinephrine
(http://me.hawkelibrary.com)
Physiology


(http://flylib.com/books/en/2.953.1.20/1/)
Temporal Bone Paraganglia
Oxygen baroreceptors
Cell clusters

Type I





Chief cells
Catecholamines
Dark cell type
Light cell type
Type II


Sustentacular cells
Modified Schwann cells
Type I cell
(Rao 1999)
Type II cell
Physiology
Temporal Bone Paraganglia

Third branchial arch (Zak 1982)




Two or three present, maybe more in fifth decade
Nonchromaffin, lack chromium salt affinity
Distinction from adrenal neuroendocrine
system
Anterior Jugular
Paraganglioma
Location

Two typical regions





Tympanic
Paraganglioma
Posterior Jugular
Paraganglioma
Anterolateral jugular fossa
Within middle ear
Jugular bulb adventitia
Jacobson nerve (cranial nerve IX)
Arnold nerve (cranial nerve X)
(Swartz 2009)
Physiology
Temporal Bone Paraganglioma

Third branchial arch (Zak 1982)




Two or three present, maybe more in fifth decade
Nonchromaffin, lack chromium salt affinity
Distinction from adrenal neuroendocrine
system
Location

Two typical regions





Anterolateral jugular fossa
Within middle ear
Jugular bulb adventitia
Jacobson nerve (cranial nerve IX)
Arnold nerve (cranial nerve X)


Glomus jugulare
Glomus tympanicum
Extratemporal Paraganglioma

Carotid body tumor

Glomus vagale
Temporal Paraganglioma
Epidemiology


First described by Gould in 1941 (O’Leary 1991)
Most common true neoplasm of middle ear




More frequent in Caucasians




Most common pathologic condition of jugular foramen
Second most common tumor of the temporal bone
Rare overall, 0.012% in 600,000 (Bertrand 1976, Balatsouras 1992)
Usually on left side
Female:Male ratio 6:1
Peak incidence in fifth decade
Aggressive in younger patients


Commonly multifocal
Likely to secrete vasoactive substances
(N Engl J Med 2010; 362:e66.)
Temporal Paraganglioma
Epidemiology


Multiple tumors in 5-10%
Familial autosomal dominant disorder



Less than 10% of cases
Multicentricity in 30-50% affected
Associated with defects on chromosome 11q23
(Petropoulous 2000)
Metastasis in 3-4% (Pluta 1994, Motegi 2008)





Lymph nodes
Lung
Liver
Spleen
Bone
(J Nucl Med 2012; 53:264-274.)

Temporal Paraganglioma
Characteristics
Slow-growing, firm red mass


Bleeds profusely with manipulation
Neurological deficits in advanced cases



Chief cells



Clusters, “zellballen”
Nuclear pleomorphism
and hyperchromatism
Store catecholamines


Actively secrete norepinephrine in 1-3%
More likely to be secreted by glomus jugulare
(http://me.hawkelibrary.com)

Dysphagia, dysarthria (hypoglossal canal)
Facial hypesthesia (petrous apex)
Ataxia, imbalance (posterior fossa, cerebellum)
(Rao 1999)

Temporal Paraganglioma
Symptomatology




Hearing loss (80%)
Pulsatile tinnitus (60%)
Aural fullness (18%)
Rupture through
tympanic membrane (7%)




Otalgia
Otorrhagia
Vertigo/dizziness (9%)
Headache (4%)
Temporal Paraganglioma
Symptomatology: Secreting Tumors




Hearing loss (80%)
Pulsatile tinnitus (60%)
Aural fullness (18%)
Rupture through
tympanic membrane (7%)




Otalgia
Otorrhagia
Vertigo/dizziness (9%)
Headache (4%)







Flushing
Diarrhea
Palpitations
Headache
Labile hypertension
Orthostasis
Diaphoresis
Temporal Paraganglioma
Reddish-blue mass on otoscopy





Medial to inferior tympanic membrane
Pulsatile
Blanching with positive pressure
(Brown sign)
Decreased pulsations with
carotid compression
(Aquino sign)
Audible bruit auscultated
(objective tinnitus)


Mastoid
Infra-auricular region
(http://www.explosm.net)

(http://www.youtube.com)
Physical Examination
Temporal Paraganglioma
Physical Examination



Typically conductive (52%)
Sensorineural if labyrinth invaded (5%)
Mixed hearing loss (17%)
Neurological


Facial nerve palsy
Vernet syndrome



Villaret syndrome



Jugular foramen syndrome
Cranial nerves IX, X, XI
Jugular foramen and Horner syndromes
Miosis, ptosis, anhydrosis
Ataxia
(AJNR 2002 23: 929-931)

(Arq. Neuro-Psiquiatr 2006; 64: 603-605.)
Hearing loss
(http://ptjournal.apta.org)

Pulsatile Tinnitus
Differential Diagnosis

Uncommon otologic symptom
Vascular pathophysiology



Increased vascular blood flow
Vascular lumen stenosis
Nonvascular



Sensorineural hearing loss
Superior semicircular canal dehiscence
Myoclonic contractions




Tensor veli palatini
Levator veli palatini
Salpingophayrngeus
Superior constrictor
(RadioGraphics 2006; 26:115-124)

Pulsatile Tinnitus
Arterial Etiologies


Carotid atherosclerosis
Intracranial vascular
abnormalities



Dural arteriovenous fistula (AVF)
Arteriovenous malformation (AVM)
Aneurysm





(RadioGraphics
2004; 24:1637-1653)
Anterior inferior cerebellar
artery
Internal carotid artery
Vertebral artery
Tortuous internal carotid
artery
Fibromuscular dysplasia
(J Neurol Neurosurg
Psychiatry 2004; 75:993)
(J NeuroIntervent
Surg 2010; 2:202-207)
Pulsatile Tinnitus
Venous Etiologies


(http://www.radiologyassistant.nl)

High jugular bulb
Jugular bulb dehiscence
Jugular diverticula
Abnormal condylar and mastoid
emissary veins
(Int J Pediatr Otorhinolaryngol
2012; 76:447-451)

(Indian Journal of Otology
2011; 17:123-126)

Idiopathic intracranial
hypertensive syndrome (IIH)
Jugular bulb abnormalities
(Radiology 2000; 216:342-349)

Pulsatile Tinnitus
Physical Examination


Otoscopy
Inspect oral cavity and pharynx for contractions



Venous pulsatile tinnitus



Wide oral opening may decrease contractions
Fiberoptic nasopharyngoscopy
Decreases with internal jugular vein compression
Decreases with head rotation ipsilaterally
Neurological
Pulsatile Tinnitus
Diagnostics


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Audiological
Carotid Duplex ultrasound
Computed tomography
angiography (CTA)
Magnetic resonance
venography
Carotid angiography if
suspect AVF/AVM
Lumbar puncture if suspect IIH
CTA temporal bone and neck if abnormal otoscopy
(Sismanis 2011)

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Computed tomography (CT)
Magnetic resonance imaging (MRI)
Angiography
Serum catecholamines
Urinary vanillylmandelic acid
Urinary metanephrine
Positron emission tomography
(Hoegerle 2003)

Octreotide scintigraphy (Bustillo 2004)
(http://emedicine.medscape.com)
(http://me.hawkelibrary.com)
Diagnostics
(RadioGraphics 2009; 29:1877-1896)
Temporal Paraganglioma
Diagnostics
CT

Bony partition between jugular fossa and hypotympanum



Glomus jugulare erodes jugular fossa
Glomus tympanicum occupies middle ear
Jugular foramen


Enlargement if length + width greater than 20mm
Bony erosion with decalcification
Glomus Jugulare
(Rao 1999)
(Swartz 2009)
(http://me.hawkelibrary.com)
Glomus Tympanicum
Diagnostics
CT

Caroticojugular spine





Separates jugular bulb from
petrous carotid artery
Erosion with glomus jugulare
Intracranial extension
Invasion of fallopian canal
Other vascular abnormalities


High jugular bulb
Aberrant carotid artery
(Rao 1999)
(Imaging 2007; 19:55-70)
(Radiology Case Reports 2009; 4(4))
(http://me.hawkelibrary.com)
Diagnostics
CT Differential: Schwannoma
(AJNR 2000; 21:1139-1144)

Smooth erosion of jugular foramen
Glomus erosion typically irregular margins
(AJR 2004; 182:373-377)

Diagnostics
CT Differential: Meningioma


May be difficult to distinguish from
schwannomas
Tend to infiltrate bone around
jugular foramen
(Indian J Pathol Microbiol 2011;54:398-9)
(Iran J Radiol 2011;8:176-81)
(http://www.medscape.com)
Diagnostics
MRI

Vascular




Intracranial extension




“Salt and pepper” flow voids
Intraluminal involvement of
petrous carotid artery
Occlusion of jugular vein and
sigmoid sinus
Intradural
Extradural
Screen for multiple tumors
Assess for glomus vagale tumor
(http://sumerdoc.blogspot.com)
Diagnostics
MRI Differential: Schwannoma

Smooth contoured mass
T1-weighted images



Iso-intense without contrast
Significant gadolinium enhancement
High signal intensity on T2-weighted images
(http://radiopaedia.org)

Diagnostics
MRI Differential: Meningioma

T1- and T2-weighted imaging



Iso-intense to grey matter
Increased intensity with contrast
“Dural tails”
(Iran J Radiol 2011;8:176-81)
(Surgical Neurology 2001; 56:8-20)
Diagnostics
CT versus MRI

CT usually sufficient imaging alone



MRI when diagnosis or extent is questioned





Jugular bulb major preoperative consideration
Supplants venography to assess jugular bulb
Delineates neoplastic and native tissue
Good for intradural tumors
T1 images may overestimate
tumor extent (Brackmann 2010)
Petrous apex best imaged with
CT and MRI (Arriaga 1991)
Magnetic resonance angiography
typically inadequate
Diagnostics
Angiography



Better assess larger tumors
Identify multicentric disease
Preoperative embolization





Performed 1-2 days before surgery
Polyvinyl alcohol or intravascular coils
Decrease intraoperative blood loss
Balloon occlusion if anticipate
carotid sacrifice
Carotid sacrifice morbidity (Linskey 1994)


Cerebral infarction 26%
Fatality 46%
(http://www.aneurysm-stroke.com)
Diagnostics
Angiography



Better assess larger tumors
Identify multicentric disease
Preoperative embolization





Performed 1-2 days before surgery
Polyvinyl alcohol or intravascular coils
Decrease intraoperative blood loss
Balloon occlusion if anticipate
carotid sacrifice
Carotid sacrifice morbidity (Linskey 1994)


Cerebral infarction 26%
Fatality 46%
Before Embolization
(http://me.hawkelibrary.com)
After Embolization
Angiography
Arterial Supply

Primary supply


Inferior tympanic artery (from
ascending pharyngeal artery)
Stylomastoid artery



Ascending
Pharyngeal
Occipital artery (60%)
Posterior auricular artery (40%)
Other contributors (Hesselink 1981)



Middle meningeal artery
Internal carotid artery
External carotid artery
Contributors

Internal maxillary

Middle meningeal

Posterior auricular
(AJNR 2006; 27:1820-1822)
Angiography
Arterial Supply

Vascular compartments `
(Moret 1980, Moret 2982, Russel 1986, Young 1988)






Inferomedial
Posterolateral
Anterior
Superior
Each compartment
hemodynamically
independent
Multicompartmental in
85% glomus tumors
Contributors

Occipital

Posterior auricular
(http://www.interventionalneuroradiology.net)
Angiography
Arterial Supply
Compartment
Location
Blood Supply
Inferomedial
Jugular bulb,
Hypotympanum
Inferior tympanic branch of
ascending pharyngeal
Posterolateral
Posterior tympanic cavity,
Mastoid
Anterior
Protympanum,
Pericarotid area
Superior
Epitympanum,
Supralabyrinthine
(Alerstone 1996)
Stylomastoid
Anterior tympanic branch
of internal maxillary,
Caroticotympanic branch
of internal carotid
Superior tympanic branch
of middle meningeal artery
Glomus Tumors
Pattern of Spread

“Danger zone” around jugular bulb (Minor 1994)







Inferior petrosal sinus
Internal jugular vein
Sigmoid sinus
Protympanum
Hypotympanum
Mesotympanum
Intracranial extension


Radiograhic diagnosis 14-20% (Rigby 1996)
Up to 50% with dural involvement intraoperatively
(Spector 1976, Andrews 1989, Jackson 1990)
(Minor 1994)
Patterns of Spread
Protympanum
Peritubal cell tract

Petrous apex
Carotid canal

Middle cranial fossa

Most common route for
intracranial extension via
protympanum
Eustachian tube

Nasopharyngeal mass

Epistaxis
Facial
Nerve
(Minor 1994)
Jugular
Vein
Internal
Carotid
Patterns of Spread
Hypotympanum
Inferior petrosal sinus
Sigmoid sinus lumen
Neural foramina
at skull base
Internal jugular lumen
Facial
Nerve
(Minor 1994)
Jugular
Vein
Internal
Carotid
Patterns of Spread
Mesotympanum
Round window

Erode cochlea

Internal auditory canal
Antrum and
epitympanum



Facial recess
Sinus tympani
Mastoid
Laterally

External auditory canal
Facial
Nerve
(Minor 1994)
Jugular
Vein
Internal
Carotid
Classification
Glasscock-Jackson
GLOMUS TYMPANICUM
TYPE
CHARACTERISTICS
I
Limited to the promontory
II
Completely fills the middle ear space
III
Fills the middle ear and extends to mastoid
IV
Extend into external auditory canal
May extend anterior to internal carotid artery
Classification
Glasscock-Jackson
GLOMUS JUGULARE
TYPE
CHARACTERISTICS
I
Involves jugular bulb, middle ear, and mastoid
II
Extends underneath internal auditory canal
May have intracranial extension
III
Extends into petrous apex
May have intracranial extension
IV
Extends into clivus and infratemporal fossa
May have intracranial extension
Classification
Fisch
GLOMUS TUMORS
TYPE
CHARACTERISTICS
A
Limited to middle ear cleft
B
Limited to tympanomastoid complex
No infralabyrinthine involvement
C
Involves labyrinthine compartment
Extends to petrous apex
D
Intracranial involvement
Classification
Fisch
GLOMUS TUMORS
TYPE
CHARACTERISTICS
A
Limited to middle ear cleft
B
Limited to tympanomastoid complex
No infralabyrinthine involvement
C
Involves labyrinthine compartment
Extends to petrous apex
D
Intracranial involvement
(Fisch 1988)
Classification
Fisch
GLOMUS TUMORS
TYPE
A
CHARACTERISTICS
Limited to middle ear cleft
(Fisch 1988)
B
Limited to tympanomastoid complex
No infralabyrinthine involvement
C
Involves labyrinthine compartment
Extends to petrous apex
D
Intracranial involvement
Classification
Fisch
TYPE
CHARACTERISTICS
GLOMUS TUMORS
Limited involvement of
C1
TYPE
CHARACTERISTICS
vertical portion of carotid
canal
Invades vertical portion
C2 A
Limited
of carotid
canalto middle ear cleft
C3
Invades horizontal portion
Limited to tympanomastoid complex
B of carotid canal
No infralabyrinthine involvement
C
Involves labyrinthine compartment
Extends to petrous apex
D
Intracranial involvement
(Fisch 1988)
Classification
Fisch
TYPE
CHARACTERISTICS
GLOMUS TUMORS
Limited involvement of
C1
TYPE
CHARACTERISTICS
vertical portion of carotid
canal
Invades vertical portion
C2 A
Limited
of carotid
canalto middle ear cleft
C3
Invades horizontal portion
Limited to tympanomastoid complex
B of carotid canal
No infralabyrinthine involvement
C
Involves labyrinthine compartment
Extends to petrous apex
D
Intracranial involvement
(Fisch 1988)
Classification
Fisch
TYPE
CHARACTERISTICS
GLOMUS TUMORS
Limited involvement of
C1
TYPE
CHARACTERISTICS
vertical portion of carotid
canal
Invades vertical portion
C2 A
Limited
of carotid
canalto middle ear cleft
C3
Invades horizontal portion
Limited to tympanomastoid complex
B of carotid canal
No infralabyrinthine involvement
C
Involves labyrinthine compartment
Extends to petrous apex
D
Intracranial involvement
(Fisch 1988)
Classification
Fisch
TYPE
CHARACTERISTICS
GLOMUS TUMORS
Limited involvement of
C1
TYPE
CHARACTERISTICS
vertical portion of carotid
canal
Invades vertical portion
C2 A
Limited
of carotid
canalto middle ear cleft
C3
Invades horizontal portion
Limited to tympanomastoid complex
B of carotid canal
No infralabyrinthine involvement
C
Involves labyrinthine compartment
Extends to petrous apex
D
Intracranial involvement
(Fisch 1988)
Classification
Fisch
GLOMUS TUMORS
TYPE
A
TYPE
CHARACTERISTICS
Limited to middle ear cleft
CHARACTERISTICS
D1 B
Limited to tympanomastoid complex
Intracranial extension less than 2cm
No infralabyrinthine involvement
D2C
Involvesextension
labyrinthine
Intracranial
greatercompartment
than 2cm
D
Extends to petrous apex
Intracranial involvement
Classification
Fisch
GLOMUS TUMORS
TYPE
A
TYPE
CHARACTERISTICS
Limited to middle ear cleft
CHARACTERISTICS
D1 B
Limited to tympanomastoid complex
Intracranial extension less than 2cm
No infralabyrinthine involvement
D2C
Involvesextension
labyrinthine
Intracranial
greatercompartment
than 2cm
D
Extends to petrous apex
Intracranial involvement
(Fisch 1988)
Classification
Fisch
GLOMUS TUMORS
TYPE
A
TYPE
CHARACTERISTICS
Limited to middle ear cleft
CHARACTERISTICS
D1 B
Limited to tympanomastoid complex
Intracranial extension less than 2cm
No infralabyrinthine involvement
D2C
Involvesextension
labyrinthine
Intracranial
greatercompartment
than 2cm
D
Extends to petrous apex
Intracranial involvement
(Fisch 1988)
Temporal Paraganglioma
Treatment

Excision





Definitive treatment in 90%
Staged for larger tumors
Laser or bipolar cautery for excision
and hemostasis
Alpha- and beta- blockade
secreting tumors
Radiation


Adjuvant therapy if incomplete
resection
Poor or unwilling surgical
candidates
Treatment
Surgery

Transcanal approach






Small tumors on promontory
Drill canal for hypotympanum access
Facial recess approach for middle ear
and mastoid
Transmastoid-transcervical for glomus
jugulare
Modified infratemporal approach for
larger tumors
Unresectability (Jackson 1982, Rufini 2006)


Foramen magnum
Cavernous sinus
(Minor 1994)
Treatment
Transmastoid-Transcervical
Complete mastoidectomy
Open facial recess
Identify vasculature
and nerves
(Minor 1994)
Treatment
Transmastoid-Transcervical
Amputate mastoid tip and
ligate internal jugular vein
Pack sigmoid sinus and
transpose facial nerve
(Minor 1994)
Treatment
Fisch Surgical Approach




Postauricular approach to infratemporal fossa
Type A
Type B
Type C
(Snow 2009)
Treatment
Fisch Surgical Approach




Postauricular approach to infratemporal fossa
Type A

Radical mastoidectomy
Type B

Anterior transposition of facial nerve
Type C


Explore posterior infratemporal fossa
Cervical dissection



Jugular bulb
Vertical petrous carotid
Posterior infratemporal fossa
Treatment
Fisch Surgical Approach




Postauricular approach to infratemporal fossa
Type A

Explore petrous apex
Type B

Mid-clivus
Type C


Horizontal internal carotid artery
Superior infratemporal fossa
Treatment
Fisch Surgical Approach




Postauricular approach to infratemporal fossa
Type A

Nasopharynx
Type B

Peritubal space
Type C






Rostral clivus
Parasellar area
Cavernous sinus
Pterygopalatine fossa
Anterosuperior infratemporal fossa
Foramen rotundum
Treatment
Modified Infratemporal Approach
Similar postauricular
incision

External auditory canal (EAC)
is transected and oversewn

EAC, tympanic membrane,
midldle ear contents resected
Facial nerve mobilized
(Canalis 2000)
(Glasscock 2003)
Treatment
Modified Infratemporal Approach

Anterosuperior extension

Resect zygoma and
temporomandibular joint

Reflect temporalis inferiorly

Dislocate mandibular
anteroinferiorly


Resect eustachian tube
Expose internal carotid artery
Access
ο
ο
ο
ο
ο
Middle and posterior cranial fossa
Nasopharynx
Foramen rotundum
Clivus
(Canalis 2000)
Cavernous sinus
(Glasscock 2003)
Treatment
Transcondylar/Suboccipital


Intracranial tumors near foramen
magnum
Supplement intratemporal approach



Trans-sigmoid exposure
Trans-labyrinthine exposure
Exposures cranial cervical junction
(Brackmann 2010)
Treatment
Transcondylar/Suboccipital

Identify vertebral artery



Expose occipital condyle and
jugular tubercle



Posterior and inferior to mastoid tip
On transverse process of C1
vertebra
Access hypoglossal nerve
Access cranial cervical junction
Cervical stabilization procedure
if more than half of condyle
resected
(Brackmann 2010)
Treatment
Surgical Considerations
Cerebellum


PICA
Leave tumor portion if adherent
to internal carotid
Cranial nerve preservation
enhanced if medial wall of
jugular bulb preserved
Intradural involvement



Commonly at jugular bulb
En-bloc resection if blood loss less
than 2000mL
Otherwise staged procedures
(Brackmann 2010)

Pons
PICA – posterior inferior cerebellar artery
Treatment
Surgical Complications



Bleeding
Facial paresis/paralysis
Cranial nerve palsy






Hoarseness
Dysphagia
Dysarthria
Cerebrospinal fluid leak
Tympanic membrane
perforation (O’Leary 1989, Forest 2001)
Cholesteatoma (O’Leary 1989, Forest 2001)
Treatment
Radiotherapy



External beam
Stereotactic
Not ablative



Obliterative endarteritis in tumor vessels
stops growth
Tumor control in over 90% (Maarouf 2003, Krych 2006)
Regrowth possible after 10-15 years
(Brackmann 2010)

Comparable to surgery



Tumor control
Recurrence
Morbidity
Treatment
Radiotherapy

Indications





Dual-modality treatment with
surgery



Elderly
Poor surgical candidates
Some multicentric lesions
Patient preference
Limit cranial neuropathies
Adjuvant therapy after near-total
resection
No long-term data
Treatment
Radiotherapy

Surveillance (Swartz 2009)






Residual mass
Stabile or decreased size
Decreased enhancement on CT
Reduced T2-weighted signal on MRI
Diminished flow voids
Morbidity




Cranial nerve deficit
Osteoradionecrosis
Tumor regrowth
Radiation-induced malignancy
(Lustig 1997)
Conclusion
Glomus Tumors of Temporal Bone




Most common tumor of middle
ear but rare
Pulsatile tinnitus and hearing
loss classic symptomatology
Characteristic radiographical
features
Surgery most definitive treatment



Transcanal for small tumors
Transmastoid-transcervical for larger
ones
Radiation acceptable alternative
for nonsurgical candidates
(http://www.smbc-comics.com)
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