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 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) 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) References Alberstone CD, Anson JA. 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