Download - Glomus Tumors of the Temporal Bone: Synopsis of Glomus ...

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

Glomus Tumors

of the Temporal Bone

All images obtained via Google search unless otherwise

specified. All images used without permission.

Viet Pham MD

Dayton Young MD

Synopsis of Glomus Tympanicum and Jugulare

(http://www.explosm.net)

May 29, 2012

Paraganglia

Paraganglioma

Presentation

Diagnostics

Classification

Treatment

Outline

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

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

Physiology Paraganglia

(http://me.hawkelibrary.com)

Oxygen baroreceptors

Cell clusters

Type I

Chief cells

Catecholamines

Dark cell type

Light cell type

Type II

Sustentacular cells

Modified Schwann cells

Physiology Temporal Bone Paraganglia

(http://f

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Type I cell Type II cell (Rao 1999)

Physiology Temporal Bone Paraganglia

Tympanic

Paraganglioma

Anterior Jugular

Paraganglioma

Posterior Jugular

Paraganglioma

(Swartz 2009)

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)

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)

Physiology Temporal Bone Paraganglioma

Glomus jugulare

Glomus tympanicum

Extratemporal Paraganglioma

Carotid body tumor

Glomus vagale

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

Temporal Paraganglioma Epidemiology

(N Engl J Med 2010; 362:e66.)

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

Temporal Paraganglioma Epidemiology

(J N

ucl M

ed

2012; 53:2

64-2

74.)

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

and hyperchromatism

Store catecholamines

Actively secrete norepinephrine in 1-3%

More likely to be secreted by glomus jugulare

Temporal Paraganglioma Characteristics

(http://m

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(Rao 1

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Temporal Paraganglioma Symptomatology

Hearing loss (80%)

Pulsatile tinnitus (60%)

Aural fullness (18%)

Rupture through

tympanic membrane (7%)

Otalgia

Otorrhagia

Vertigo/dizziness (9%)

Headache (4%)

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

Flushing

Diarrhea

Palpitations

Headache

Labile hypertension

Orthostasis

Diaphoresis

Temporal Paraganglioma Physical Examination

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://w

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Hearing loss

Typically conductive (52%)

Sensorineural if labyrinth invaded (5%)

Mixed hearing loss (17%)

Neurological

Facial nerve palsy

Vernet syndrome

Jugular foramen syndrome

Cranial nerves IX, X, XI

Villaret syndrome

Jugular foramen and Horner syndromes

Miosis, ptosis, anhydrosis

Ataxia

Temporal Paraganglioma Physical Examination

(AJN

R 2

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(Arq

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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

Pulsatile Tinnitus Differential Diagnosis

(Radio

Gra

phic

s 2

006; 26:1

15

-124)

Carotid atherosclerosis

Intracranial vascular

abnormalities

Dural arteriovenous fistula (AVF)

Arteriovenous malformation (AVM)

Aneurysm

Anterior inferior cerebellar

artery

Internal carotid artery

Vertebral artery

Tortuous internal carotid

artery

Fibromuscular dysplasia

Pulsatile Tinnitus Arterial Etiologies

(J Neurol Neurosurg

Psychiatry 2004; 75:993)

(J NeuroIntervent

Surg 2010; 2:202-207)

(RadioGraphics

2004; 24:1637-1653)

Idiopathic intracranial

hypertensive syndrome (IIH)

Jugular bulb abnormalities

High jugular bulb

Jugular bulb dehiscence

Jugular diverticula

Abnormal condylar and mastoid

emissary veins

Pulsatile Tinnitus Venous Etiologies

(http://w

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Pulsatile Tinnitus Physical Examination

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

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

Pulsatile Tinnitus Diagnostics

(Sismanis 2011)

Computed tomography (CT)

Magnetic resonance imaging (MRI)

Angiography

Serum catecholamines

Urinary vanillylmandelic acid

Urinary metanephrine

Positron emission tomography (Hoegerle 2003)

Octreotide scintigraphy (Bustillo 2004)

Temporal Paraganglioma Diagnostics

(http://m

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rary

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(http://emedicine.medscape.com)

(Radio

Gra

phic

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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

Diagnostics CT

Glomus Tympanicum Glomus Jugulare

(Rao 1999)

(Swartz 2009)


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

Smooth erosion of jugular foramen

Glomus erosion typically irregular margins

Diagnostics CT Differential: Schwannoma

(AJR

2004; 182:3

73

-377)

(AJN

R 2

000; 21:1

139-1

144)

May be difficult to distinguish from

schwannomas

Tend to infiltrate bone around

jugular foramen

Diagnostics CT Differential: Meningioma

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

Vascular

“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

Diagnostics MRI

(http://sumerdoc.blogspot.com)

Smooth contoured mass

T1-weighted images

Iso-intense without contrast

Significant gadolinium enhancement

High signal intensity on T2-weighted images

Diagnostics MRI Differential: Schwannoma

(http://r

adio

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)

T1- and T2-weighted imaging

Iso-intense to grey matter

Increased intensity with contrast

“Dural tails”

Diagnostics MRI Differential: Meningioma

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

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

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%

Diagnostics Angiography

(http://www.aneurysm-stroke.com)

http://www.aneurysm-stroke.com/embolization.php

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%

Diagnostics Angiography

Before Embolization After Embolization


http://www.aneurysm-stroke.com/embolization.php

Primary supply

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

Angiography Arterial Supply

(AJNR 2006; 27:1820-1822)

Ascending

Pharyngeal

Contributors

Internal maxillary

Middle meningeal

Posterior auricular

Vascular compartments ` (Moret 1980, Moret 2982, Russel 1986, Young 1988)

Inferomedial

Posterolateral

Anterior

Superior

Each compartment

hemodynamically

independent

Multicompartmental in

85% glomus tumors


(http://www.interventionalneuroradiology.net)

Contributors

Occipital

Posterior auricular


Compartment Location Blood Supply

Inferomedial Jugular bulb,

Hypotympanum

Inferior tympanic branch of

ascending pharyngeal

Posterolateral Posterior tympanic cavity,

Mastoid Stylomastoid

Anterior Protympanum,

Pericarotid area

Anterior tympanic branch

of internal maxillary,

Caroticotympanic branch

of internal carotid

Superior Epitympanum,

Supralabyrinthine

Superior tympanic branch

of middle meningeal artery

(Alerstone 1996)

(Minor 1994)

“Danger zone” around jugular bulb (Minor 1994)

Inferior petrosal sinus

Internal jugular vein

Sigmoid sinus

Protympanum

Hypotympanum

Mesotympanum


Radiograhic diagnosis 14-20% (Rigby 1996)

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

Glomus Tumors Pattern of Spread

Patterns of Spread Protympanum

Facial

Nerve

Jugular

Vein Internal

Carotid

Carotid canal

Middle cranial fossa

Most common route for

intracranial extension via

protympanum

Eustachian tube

Nasopharyngeal mass

Epistaxis

Peritubal cell tract

Petrous apex

(Minor 1994)

Patterns of Spread Hypotympanum

Facial

Nerve

Jugular

Vein Internal

Carotid

Sigmoid sinus lumen

Internal jugular lumen

Inferior petrosal sinus

Neural foramina

at skull base

(Minor 1994)

Patterns of Spread Mesotympanum

Facial

Nerve

Jugular

Vein Internal

Carotid

Antrum and

epitympanum

Laterally

External auditory canal

Round window

Erode cochlea

Internal auditory canal

(Minor 1994)

Facial recess

Sinus tympani

Mastoid

Classification Glasscock-Jackson

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

GLOMUS TYMPANICUM

Classification Glasscock-Jackson


I Involves jugular bulb, middle ear, and mastoid

II Extends underneath internal auditory canal

May have intracranial extension

III Extends into petrous apex


IV Extends into clivus and infratemporal fossa


GLOMUS JUGULARE

Classification Fisch


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

GLOMUS TUMORS









GLOMUS TUMORS

(Fisch 1988)








GLOMUS TUMORS



C1 Limited involvement of

vertical portion of carotid canal

C2 Invades vertical portion

of carotid canal

C3 Invades horizontal portion

of carotid canal (Fisch 1988)








GLOMUS TUMORS



C1 Limited involvement of

vertical portion of carotid canal

C2 Invades vertical portion

of carotid canal

C3 Invades horizontal portion

of carotid canal

(Fisch 1988)

GLOMUS TUMORS










D1 Intracranial extension less than 2cm

D2 Intracranial extension greater than 2cm

GLOMUS TUMORS










D1 Intracranial extension less than 2cm

D2 Intracranial extension greater than 2cm

(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

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

Treatment Surgery

(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

(Minor 1994)

Pack sigmoid sinus and

transpose facial nerve

Postauricular approach to infratemporal fossa

Type A

Type B

Type C

Treatment Fisch Surgical Approach

(Snow 2009)


Type A

Type B

Type C


Radical mastoidectomy

Anterior transposition of facial nerve

Explore posterior infratemporal fossa

Cervical dissection

Jugular bulb

Vertical petrous carotid

Posterior infratemporal fossa


Type A

Type B

Type C


Explore petrous apex

Mid-clivus

Horizontal internal carotid artery

Superior infratemporal fossa


Type A

Type B

Type C


Nasopharynx

Peritubal space

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

Resect eustachian tube

Expose internal carotid artery

Access

ο Middle and posterior cranial fossa

ο Nasopharynx

ο Foramen rotundum

ο Clivus

ο Cavernous sinus (Canalis 2000)

(Glasscock 2003)

Anterosuperior extension

Resect zygoma and

temporomandibular joint

Reflect temporalis inferiorly

Dislocate mandibular

anteroinferiorly

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)

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

Treatment Surgical Considerations

Cerebellum PICA Pons

(Bra

ckm

ann 2

010)

PICA – posterior inferior cerebellar artery

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 Surgical Complications

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


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)


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

Conclusion Glomus Tumors of Temporal Bone

(http://www.smbc-comics.com)

References

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Russel EJ. Functional angiography of the head and neck. AJNR 1986; 7:927-936.

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Sismanis A. Pulsatile tinnitus: contemporary assessment and management. Curr Opin Otolaryngol Head Neck Surg 2011; 19:348-357.

Snow JB, Wackym PA, Ballenger JJ. Otorhinolaryngology, 17th Ed. Shelton: BC Decker 2009. pp 427-431.

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