Glomus Tumor PDF

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)
Dayton Young
All images obtained via Google search unless otherwise
specified. All images used without permission.
MD
Outline
 Paraganglia
(http://www.dizziness-and-balance.com)
 Paraganglioma
 Presentation
 Diagnostics
 Classification
 Treatment
(http://me.hawkelibrary.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
Temporal Bone Paraganglia
(http://flylib.com/books/en/2.953.1.20/1/)
 Oxygen baroreceptors
 Cell clusters
 Type I
 Chief cells
 Catecholamines
 Dark cell type
 Light cell type
 Type II
 Sustentacular cells
 Modified Schwann cells
(Rao 1999)
Type I cell 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 Tympanic
Paraganglioma
 Distinction from adrenal neuroendocrine
system
Anterior Jugular
 Location Paraganglioma
 Two typical regions

Posterior Jugular
 Anterolateral jugular fossa Paraganglioma
 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  Glomus jugulare
 Within middle ear  Glomus tympanicum
 Jugular bulb adventitia
Extratemporal Paraganglioma
 Jacobson nerve (cranial nerve IX)  Carotid body tumor
 Arnold nerve (cranial nerve X)  Glomus vagale
Temporal Paraganglioma
Epidemiology
 First described by Gould in 1941 (O’Leary 1991)

 Most common true neoplasm of middle ear
 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)
 More frequent in Caucasians
 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.)
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
(J Nucl Med 2012; 53:264-274.)

 Lung
 Liver
 Spleen
 Bone
Characteristics
 Slow-growing, firm red mass

 Bleeds profusely with manipulation
 Neurological deficits in advanced cases
 Dysphagia, dysarthria (hypoglossal canal)
 Facial hypesthesia (petrous apex)
 Ataxia, imbalance (posterior fossa, cerebellum)
 Chief cells
 Clusters, “zellballen”
 Nuclear pleomorphism
(http://me.hawkelibrary.com)
(Rao 1999)
and hyperchromatism
 Store catecholamines
 Actively secrete norepinephrine in 1-3%
 More likely to be secreted by glomus jugulare
Symptomatology
 Hearing loss (80%)

 Pulsatile tinnitus (60%)
 Aural fullness (18%)
 Rupture through
tympanic membrane (7%)
 Otalgia
 Otorrhagia
 Vertigo/dizziness (9%)
 Headache (4%)
Symptomatology: Secreting Tumors
 Hearing loss (80%)  Flushing

 Pulsatile tinnitus (60%)  Diarrhea
 Aural fullness (18%)  Palpitations
 Rupture through  Headache
tympanic membrane (7%)  Labile hypertension
 Otalgia
 Orthostasis
 Otorrhagia
 Diaphoresis
 Vertigo/dizziness (9%)
 Headache (4%)
Physical Examination
 Reddish-blue mass on otoscopy
(http://www.youtube.com)
 Medial to inferior tympanic membrane
 Pulsatile
 Blanching with positive pressure
(Brown sign)
 Decreased pulsations with
carotid compression
(Aquino sign)
 Audible bruit auscultated
(http://www.explosm.net)
(objective tinnitus)
 Mastoid
 Infra-auricular region
 Hearing loss
 Typically conductive (52%)
(Arq. Neuro-Psiquiatr 2006; 64: 603-605.)

 Sensorineural if labyrinth invaded (5%)
 Mixed hearing loss (17%)
 Neurological
(http://ptjournal.apta.org)
 Facial nerve palsy
 Vernet syndrome
 Jugular foramen syndrome
 Cranial nerves IX, X, XI
(AJNR 2002 23: 929-931)

 Villaret syndrome
 Jugular foramen and Horner syndromes
 Miosis, ptosis, anhydrosis
 Ataxia
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
(RadioGraphics 2006; 26:115-124)


 Tensor veli palatini

 Levator veli palatini
 Salpingophayrngeus
 Superior constrictor
Pulsatile Tinnitus
Arterial Etiologies
 Carotid atherosclerosis
 Intracranial vascular
abnormalities
 Dural arteriovenous fistula (AVF)
 Arteriovenous malformation (AVM) (RadioGraphics
2004; 24:1637-1653)
(J NeuroIntervent
Surg 2010; 2:202-207)
 Aneurysm
 Anterior inferior cerebellar
artery
 Internal carotid artery
 Vertebral artery
 Tortuous internal carotid
artery
(J Neurol Neurosurg
 Fibromuscular dysplasia Psychiatry 2004; 75:993)
Pulsatile Tinnitus
Venous Etiologies
 Idiopathic intracranial
(http://www.radiologyassistant.nl)
hypertensive syndrome (IIH)
 Jugular bulb abnormalities
 High jugular bulb
 Jugular bulb dehiscence
 Jugular diverticula
 Abnormal condylar and mastoid
emissary veins
(Int J Pediatr Otorhinolaryngol

(Radiology 2000; 216:342-349)
(Indian Journal of Otology
2012; 76:447-451)
2011; 17:123-126)
Pulsatile Tinnitus
 Otoscopy
 Inspect oral cavity and pharynx for contractions
 Wide oral opening may decrease contractions
 Fiberoptic nasopharyngoscopy
 Venous pulsatile tinnitus
 Decreases with internal jugular vein compression
 Decreases with head rotation ipsilaterally
 Neurological
Pulsatile Tinnitus
Diagnostics
 Audiological
 Carotid Duplex ultrasound
 Computed tomography
angiography (CTA)
 Magnetic resonance
venography
 Carotid angiography if (Sismanis 2011)
suspect AVF/AVM
 Lumbar puncture if suspect IIH
 CTA temporal bone and neck if abnormal otoscopy
Diagnostics
(RadioGraphics 2009; 29:1877-1896)

 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)
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
(Rao 1999)
 Bony erosion with decalcification (Swartz 2009)
Glomus Jugulare 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))
Diagnostics
CT Differential: Schwannoma
 Smooth erosion of jugular foramen

 Glomus erosion typically irregular margins
(AJNR 2000; 21:1139-1144)

(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
 “Salt and pepper” flow voids
 Intraluminal involvement of
petrous carotid artery
 Occlusion of jugular vein and
sigmoid sinus
 Intracranial extension
 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

 Jugular bulb major preoperative consideration
 Supplants venography to assess jugular bulb
 MRI when diagnosis or extent is questioned
 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 After Embolization
Angiography
Arterial Supply
 Primary supply Ascending

Pharyngeal
 Inferior tympanic artery (from
ascending pharyngeal artery)
 Stylomastoid artery
 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 Contributors
 Occipital
 Multicompartmental in
 Posterior auricular
85% glomus tumors
(http://www.interventionalneuroradiology.net)
Angiography
Arterial Supply
Compartment Location Blood Supply

Jugular bulb, Inferior tympanic branch of
Inferomedial
Hypotympanum ascending pharyngeal
Posterior tympanic cavity,

Posterolateral Stylomastoid
Mastoid
Anterior tympanic branch

Protympanum, of internal maxillary,
Anterior
Pericarotid area Caroticotympanic branch
of internal carotid
Epitympanum, Superior tympanic branch

Superior
Supralabyrinthine of middle meningeal artery
(Alerstone 1996)
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 (Minor 1994)
 Radiograhic diagnosis 14-20% (Rigby 1996)

 Up to 50% with dural involvement intraoperatively
(Spector 1976, Andrews 1989, Jackson 1990)
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 Jugular Internal

Nerve Vein Carotid
(Minor 1994)
Patterns of Spread
Hypotympanum
Inferior petrosal sinus

Sigmoid sinus lumen
Internal jugular lumen Neural foramina

at skull base

Nerve Vein Carotid
(Minor 1994)
Patterns of Spread
Mesotympanum
Round window
 Erode cochlea
Antrum and  Internal auditory canal
epitympanum
 Facial recess
 Sinus tympani
Laterally
 Mastoid
 External auditory canal

Nerve Vein Carotid
(Minor 1994)
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
Extend into external auditory canal

IV
May extend anterior to internal carotid artery
Classification
Glasscock-Jackson
GLOMUS JUGULARE
I Involves jugular bulb, middle ear, and mastoid
Extends underneath internal auditory canal

II
May have intracranial extension
Extends into petrous apex
III
Extends into clivus and infratemporal fossa
IV
Classification
Fisch
GLOMUS TUMORS
A Limited to middle ear cleft
Limited to tympanomastoid complex

B
No infralabyrinthine involvement
Involves labyrinthine compartment
C
Extends to petrous apex
D Intracranial involvement
Classification
Fisch
GLOMUS TUMORS

B
C
(Fisch 1988)
Classification
Fisch
GLOMUS TUMORS

(Fisch 1988)

B
C
Classification
Fisch
GLOMUS TUMORS
Limited involvement of
C1
vertical portion of carotid canal
Invades vertical portion
C2 A Limited
of carotid canalto middle ear cleft
Invades horizontal portion
C3 Limited to tympanomastoid complex
B of carotid canal
No infralabyrinthine involvement (Fisch 1988)

C
Classification
Fisch
GLOMUS TUMORS
C1
C2 A Limited
B of carotid canal

C
Classification
Fisch
GLOMUS TUMORS
C1
C2 A Limited
B of carotid canal

C
Classification
Fisch
GLOMUS TUMORS
C1
C2 A Limited
B of carotid canal
Involves labyrinthine compartment (Fisch 1988)
C
Classification
Fisch
GLOMUS TUMORS

D1 B Intracranial extension less than 2cm
D2C Involvesextension
Intracranial labyrinthine
greatercompartment
than 2cm
Classification (Fisch 1988)
Fisch
GLOMUS TUMORS

greatercompartment
than 2cm
Classification (Fisch 1988)
Fisch
GLOMUS TUMORS

greatercompartment
than 2cm
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 Pack sigmoid sinus and

ligate internal jugular vein transpose facial nerve
(Minor 1994)
Treatment
Fisch Surgical Approach
 Postauricular approach to infratemporal fossa

 Type A
 Type B
 Type C
(Snow 2009)
Treatment

 Type A
 Type B  Radical mastoidectomy
 Anterior transposition of facial nerve
 Type C
 Explore posterior infratemporal fossa
 Cervical dissection
 Jugular bulb
 Vertical petrous carotid
 Posterior infratemporal fossa
Treatment

 Type A
 Type B  Explore petrous apex
 Mid-clivus
 Type C
 Horizontal internal carotid artery
 Superior infratemporal fossa
Treatment

 Type A
 Type B  Nasopharynx
 Peritubal space
 Type C
 Rostral clivus
 Parasellar area
 Cavernous sinus
 Pterygopalatine fossa
 Anterosuperior infratemporal fossa
 Foramen rotundum
Treatment
Modified Infratemporal Approach
Similar postauricular
incision
 EAC, tympanic membrane,
External auditory canal (EAC) midldle ear contents resected
is transected and oversewn  Facial nerve mobilized
(Canalis 2000)
(Glasscock 2003)
Treatment
Modified Infratemporal Approach
 Resect eustachian tube

 Expose internal carotid artery
Anterosuperior extension
 Access
 Resect zygoma and ο Middle and posterior cranial fossa
temporomandibular joint
ο Nasopharynx
 Reflect temporalis inferiorly ο Foramen rotundum
 Dislocate mandibular ο Clivus
anteroinferiorly ο Cavernous sinus (Canalis 2000)
(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

 Posterior and inferior to mastoid tip
 On transverse process of C1
vertebra
 Expose occipital condyle and
jugular tubercle
 Access hypoglossal nerve
 Access cranial cervical junction
 Cervical stabilization procedure
if more than half of condyle
resected (Brackmann 2010)
Treatment
Surgical Considerations
Cerebellum Pons 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
(Brackmann 2010)
 Otherwise staged procedures
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
 Elderly
 Poor surgical candidates
 Some multicentric lesions
 Patient preference
 Dual-modality treatment with
surgery
 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 (http://www.smbc-comics.com)
 Radiation acceptable alternative

for nonsurgical candidates
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Glomus Tumors

of the Temporal Bone:

Viet Pham, M.D.

(http://me.hawkelibrary.com) (http://my.clevelandclinic.org) (Swartz 2009)

 Third branchial arch (Zak 1982)

 Two typical regions

 Within middle ear

 Third branchial arch (Zak 1982)

 First described by Gould in 1941 (O’Leary 1991)

 Multiple tumors in 5-10%

 Metastasis in 3-4% (Pluta 1994, Motegi 2008)

(J Nucl Med 2012; 53:264-274.)

 Slow-growing, firm red mass

 Hearing loss (80%)

 Hearing loss (80%)  Flushing

 Reddish-blue mass on otoscopy

(Arq. Neuro-Psiquiatr 2006; 64: 603-605.)

(AJNR 2002 23: 929-931)

 Uncommon otologic symptom

(RadioGraphics 2006; 26:115-124)

 Tensor veli palatini

(Int J Pediatr Otorhinolaryngol

(Indian Journal of Otology

(RadioGraphics 2009; 29:1877-1896)

 Octreotide scintigraphy (Bustillo 2004)

 Bony partition between jugular fossa and hypotympanum

Glomus Jugulare Glomus Tympanicum

 Smooth erosion of jugular foramen

(AJNR 2000; 21:1139-1144)

 May be difficult to distinguish from

(Indian J Pathol Microbiol 2011;54:398-9) (Iran J Radiol 2011;8:176-81) (http://www.medscape.com)

 Smooth contoured mass

 T1- and T2-weighted imaging

(Iran J Radiol 2011;8:176-81) (Surgical Neurology 2001; 56:8-20)

 CT usually sufficient imaging alone

 Better assess larger tumors

 Better assess larger tumors

 Primary supply Ascending

Compartment Location Blood Supply

Posterior tympanic cavity,

Anterior tympanic branch

Epitympanum, Superior tympanic branch

 “Danger zone” around jugular bulb (Minor 1994)

 Radiograhic diagnosis 14-20% (Rigby 1996)

Facial Jugular Internal

Inferior petrosal sinus

Internal jugular lumen Neural foramina

Facial Jugular Internal

Facial Jugular Internal

I Limited to the promontory

II Completely fills the middle ear space

III Fills the middle ear and extends to mastoid

Extend into external auditory canal

I Involves jugular bulb, middle ear, and mastoid

Extends underneath internal auditory canal

A Limited to middle ear cleft

Limited to tympanomastoid complex

A Limited to middle ear cleft

Limited to tympanomastoid complex

A Limited to middle ear cleft

Limited to tympanomastoid complex

Involves labyrinthine compartment

Involves labyrinthine compartment