Glomus tumour of middle ear and its management

1. GLOMUS TUMOUR
Dr Vineeth G
Junior Resident
Department of ENT
Government Medical college
Kottayam

2. Introduction
 It is the commonest benign tumour of middle
ear.
 It is BENIGN, SLOW GROWING,
HYPERVASCULAR tumour.
 It is so named because of its origin from
glomus bodies found over jugular bulb &
promontory.
 It also contains paraganglionic cells.

3. • Paraganglia cells are derived from the neural crest
• Term Paraganglion first used by histologist-Kohn
• Tumors of these paraganglia are divided into 2 groups:
(1)adrenal paragangliomas (90%)known as
pheochromocytomas and (2) extra-adrenal
paragangliomas(10%) located in the abdomen, chest,head and
neck regions
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4. Head and neck
paragangliomas are
classified based on
anatomic location and
include the carotid body,
jugulotympanic,
vagal,laryngeal,nasal and
orbital paragangliomas.
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5.  Histologically, they resemble carotid body.
 In middle ear paraganglia are distributed over –
1. Promontory – Along the branches of tympanic branch of
glossopharyngeal Nerve(jacobson’s nerve). or auricular br. Of
vagus(Arnold’s Nerve)
2. Dome of jugular bulb – Adventitial layer
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6. Glomus tympanicum tumor
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7. Glomus Jugulare Tumor-Arise from the dome of
jugular bulb
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8. 2/28/2017

9. SYNONYMS
 Chemodectoma
 Nonchromaffin paraganglioma
 Ganglia Tympanica
 Vascular tumour of middle ear
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10. • The term chemodectoma should basically be reserved for
tumours that originate from the chemoreceptor system.
• The paraganglia in the temporal bone do not belong to this
histological entity, in contrast with those in the carotid sinus.
• Less appropriate for lesions in the jugular foramen and in other
parts of the temporal bone
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11. • The term glomus tumour is not an accurate term either,
because the word glomus means a small circumscribed
histological structure in which arterioles connect directly with
veins.
• Its is due to the belief that the chief cells derived from
specialised pericytes similar to true cutaneous
arteriovenous(glomus) anastamosis tumours.
• But these tumours are unrelated to paragangliomas.
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12. • Extra adrenal paraganglion do not stain with chromium salts-
Non chromaffin paragangliomas.
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13. HISTORY
 1840 -Valentine described it first as Ganglia Tympanica.
 1879-Robert weir described two cases of intratympanic
vascular tumours with pulsating intact tympanic
membrane
 1902 -Guild found similarities between these tumour &
carotid body & coined the term Glomus Jugulare.
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14.  1924 - Mason was first to describe Glomus tumours as
hyperplastic glomus bodies.
 1945 -Rosenwasser was first to diagnose a patient with
glomus tumour & it’s surgical excision.
 1960- Alford and Guilford classified into Glomus
Tympanicum and Glomus jugulare
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15. 2/28/2017

16. 16

17. EPIDEMOLOGY
 Annual incidence of 1 case per 1.3 million people
 5 times more common in female.
 Autosomal Dominant inheritance.
 Gene responsible is located on chromosome – 11q23
 Age – Most commonly seen in 5th decade of life (30 and 60
years)
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18. • Glomus tumors are the most common
tumor of the middle ear
• Second to vestibular schwannoma as the
most common tumor of the temporal
bone.
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19. RULE OF 10
10 % Multicentric
10 % Familial
10 % Functional
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20. ETIOLOGY• 40% paraganglioma heritable
Classification
Paraganglioma syndrome 1-4 (SDHx Mutation)
Familial Phaeochromacytoma
Associated with neuroendocrine syndromes-NF-1, VHL,
MEN-II
Risk for malignancy
Multifocal Paraganglioma
Develop early in life
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21. PATHOGENESIS
Benign, Encapsulated, Slow growing, Highly
vascular, Locally invasive tumour that erodes
bone.
Expand within temporal bone via pathways of
least resistance – air cells , vascular lumens , skull
Base foramina & the eustachian tube.
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22.  The middle ear ossicles are commonly spared.
 Intracranial & extracranial extension occur.
 Metastases from glomus tumors occur in approximately
4% of cases. - Lung, Lymph nodes, Liver, Vertebrae, Ribs,
and Spleen
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23. HISTOPATHOLOGY
 Macroscopically – Deep red firm mass that bleeds
profusely on touch.
Microscopically – Clusters of Chief cells arranged in
nested pattern called ZELLBALLEN enclosed by
fibrous stroma with rich vascular plexus.
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24. HIST ZELLBALLEN
OLOGY
Ball of cells-German
zellballen pattern -
which nests of chief
cells (type I) are
surrounded by
sustenacular cells
(type II or supporting
cells) in a highly
vascular stroma.

25. • Guild classified glomus tumors into two types
depending on the amount of cellular and stromal
components:
1. Cellular glomus bodies - when the cellular
component is predominant
2. Vascular glomus bodies - when the
vascular stromal component predominates.
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26. Spread of tumor
• These tumors tend to expand within the temporal bone via the
pathways of least resistance, most important being
pneumatized air cell tracts of temporal bone esp peritubal
cells.
• They also invade and erode bone in a lobular fashion, but they
often spare the ossicular chain.
26

27. • Anteriorly – via eustachian tube to nasopharynx
• Inferiorly – via lumens of I J V & sigmoid sinus.
via carotid sheath to neck
• Laterally – middle ear & E A C
• Medially – labyrinth
• Intracranial spread – via hypotympanum & protympanum.
27

28. 28

29. • Initially, the skull base erodes in the region of the jugular fossa
and posteroinferior petrous bone, with subsequent extension
to the mastoid and adjacent occipital bone.
• Significant intracranial and extracranial extension may occur, as
well as extension within the sigmoid and inferior petrosal
sinuses.
• Neural infiltration is also common.
29

30. Clinical features
• The diagnosis of glomus tumors is often difficult because the
presenting signs and symptoms can be nonspecific, mimicking
other otologic and neurologic conditions.
• Average delay in diagnosis – 6 years
30

31. • By history, patients with glomus tumors typically present with
pulsatile tinnitus(80%), followed by the development of
hearing loss(60%).
• The hearing loss is usually conductive because the middle ear
is filled with tumor, although a sensorineural hearing loss may
develop if the cochlea is invaded.
31

32. • Other aural signs and symptoms are ear fullness, otorrhea,
hemorrhage, bruit, and the presence of a middle ear mass.
• Ear pain is uncommon.
• Involvement of the inner ear produces vertigo and
sensorineural hearing loss.
32

33. s
33

34. • Patients with glomus tympanicum tumors present with smaller
tumors because the middle ear becomes involved earlier than
with glomus vagale or jugulare tumors.
• On the other hand, patients with glomus jugulare tumors may
present with dysfunction of jugular foramen contents
34

35. • In clinical practice, perhaps the most common is Vernet's
syndrome (named after the French neurologist Maurice
Vernet).
• This syndrome comprises paralysis of the cranial nerves IX, X
and XI.
35

36. • In rare cases, a thrombosed sigmoid-jugular complex may give
rise to retrograde thrombosis of the inferior petrosal sinus and
eventually even to thrombosis of the cavernous sinus.
• This condition then leads to the cavernous sinus syndrome, i.e.
paralysis of cranial nerves III, IV and VI, oedema of the
conjunctiva and upper eye lid, venous congestion in the fundus
of the globe and proptosis.
36

37. • Blockage of the cerebral venous drainage
through the jugular foramen can also cause
increased intracranial pressure and give rise
to epileptic seizures.
37

38. • The presence of jugular foramen syndrome (paresis of
cranial nerves IX-XI) is pathognomonic for this tumor,
but it usually follows one year after the initial
symptoms of hearing loss and pulsatile tinnitus.
• Less commonly, glomus tumors produce facial nerve
palsy, hypoglossal nerve palsy, or Horner syndrome.
38

39. • Headache, hydrocephalus, and elevated
intracranial pressure may be produced by
intracranial extension of the tumor.
• Ataxia and brainstem symptoms may also develop.
39

40. • In about 2-4% of cases, the first or leading symptoms
are hypertension and tachycardia (pheochromocytoma
like symptoms) produced by catecholamines,
norepinephrine, or dopamine excreted by the tumor.
• Other related symptoms include headache,
perspiration, pallor, and nausea.
40

41. CLINICAL EXAMINATION
• Physical examination should include a complete
inspection of the ears, nose, throat and neck.
• Cranial nerve function must be documented accurately.
• If the patient has visual symptoms, an
ophthalmological opinion is essential.
41

42. • Careful otoscopic evaluation, preferably with an
operating microscope, is essential.
• Classically, a red tumor mass beneath an intact
tympanic membrane is seen – rising sun sign.
42

43. 43

44. • If limited to the promontory with all tumor margins visible, it
probably represents a small glomus tympanicum tumor.
• If the tumor extends beyond the level of the annulus
(especially inferiorly), its classification cannot be made visually.
44

45. 0toscopic stages
• Hypervascular stage – early stage. Dilatation of vessels of TM
& EAC
• Tympanic stage – rising sun sign
• Polypoid or haemorrhagic stage – TM destroyed. Polypoidal
bleeding mass in EAC.
45

46. The reddish mass filling the middle ear and pushing
the tympanic membrane
46

47. • A small mass seen in the floor of the middle ear may represent
the “tip of the iceberg” of a large glomus jugulare tumor that
may extensively involve the skull base.
• These tumors may blanch with positive pressure on
pneumotoscopy (Brown’s sign) or have cessation of tumor
pulsation with ipsilateral carotid artery compression (Aquino’s
sign).
47

48. • An audible bruit or evidence of vascular
pulsations during tympanometry may also be
present in these patients.
• A glomus tumor filling the middle ear may also
cause a diffusely discolored appearance of the
tympanic membrane, which may be mistaken for
serous fluid.
48

49. CLASSIFICATION
 LUNDGREN CLASSIFICATION
 GLASSCOCK- JACKSON CLASSIFICATION
 FISCH CLASSIFICATION
 GUILD HISTOLOGICAL CLASSIFICATION
 MODIFIED DE LA CRUZ CLASSIFICATION
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50. LUNDGRENS CLASSIFICATION
Glomus Tympanicum
Glomus Jugulare
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51. GLASSCOCK-JACKSON CLASSIFICATION
 GLOMUS TYMPANICUM :
Type I : Small tumor limited to Promontory.
Type II: Tumor completely filling Middle Ear Space.
Type III: Tumor filling middle ear & extending into Mastoid process.
Type IV: Tumor filling middle ear, extending into mastoid or through
tympanic membrane to fill external auditory canal, may extend
anterior to internal carotid artery
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52. • GLOMUS JUGULARE
Type I : Small tumor involving the jugular bulb, middle ear and
mastoid.
Type II: Tumor extending under the Internal Auditory
Canal. There may be intracranial extension.
Type III: Tumor extending into the Petrous Apex. There may be
intracranial extension.
Type IV: Tumor extending beyond the petrous apex into the
clivus and Infratemporal Fossa. There may be intracranial
extension.
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53. FISCH AND MATTOX CLASSIFICATION
 Type A - Tumor limited to Middle Ear (carries the best
prognosis)
Type B - Tumor limited to the Tympanomastoid Area
with no infralabyrinthine compartment involvement
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54. • Type C - Tumor involving the Infralabyrinthine
Compartment of temporal bone with extension to
petrous apex
Type C1 - Tumor with limited involvement of the
vertical portion of the carotid canal
Type C2 - Tumor invading the vertical portion of the
carotid canal
Type C3 - Tumor invasion of the horizontal portion of
the carotid canal2/28/2017

55. Type D - Tumor with Intracranial Extension
Type D1 - Tumor with an intracranial extension less
than 2 cm in diameter
Type D2 - Tumor with an intracranial extension
greater than 2 cm in diameter
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56. De La Cruz Glomus Tumor Classification with Associated
Surgical Approach
Classification Surgical Approach
• Tympanic - Transcanal
• Tympanomastoid - Mastoid
• Jugular bulb – Mastoid / neck (possible limited facial nerve
rerouting)
• Carotid artery - Infratemporal fossa ± subtemporal
or
Transdural Infratemporal fossa/intracranial
• Craniocervical - Transcondylar
• Vagal - Cervical
56

57. Differential diagnosis
• DIFFERENTIAL DIAGNOSIS
 Dehiscent jugular Bulb
 Otitis Media
 Otosclerosis
 Cholesterol Granuloma
 Aberrant Intrapetrous Internal Carotid Artery
 Idiopathic Hemotympanum
 Aneurysm
 Arteriovenous Malformation
 Persistent stapedial artery

58. Differential diagnoses also include
• Chordoma
• Eosinophilic Granuloma
(Histiocytosis X)
• Meningioma
• Schwannoma
• Neurofibroma
• Chondrosarcoma
• Carcinoma (primary and
metastatic)
• Cholesteatoma
• Osteoma
• Chronic mastoiditis
• Lymphoma
58

59. MANAGEMENT
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60. INVESTIGATIONS
• Any attempt at biopsy should be deferred until a complete
workup is done.
• A systematic protocol allows one to make the diagnosis and to
define the extent of the tumor.
60

61. ENDOCRINOLOGICAL TESTING
• 1-3 percent of glomus tumours have the capacity to synthesize
and secrete catecholamines- noradrenaline and dopamine.
• 24-hour urine collections for catecholamine metabolite
assessment
• The level of vanillylmandelic acid (VMA) and metanephrine in
a 24-hour urine sample may indicate neurosecretory activity
61

62. AUDIOLOGICAL EVALUATION
• Audiologic examination reveals mixed conductive and
sensorineural hearing loss.
• The sensorineural component tends to be more significant
with larger tumors.
62

63. X - RAY
• Plain skull radiography may show
1) Enlargement of the lateral jugular foramen and fossa.
2)Clouding of mastoid air cells
3)Bone destruction
4)Phelp s sign – absence of normal crest of bone between carotid
canal and jugular fossa on lateral x- ray
63

64. C T SCAN
• High Resolution thin section CT SCAN(<1mm) in both axial and
coronal plain is the imaging modality of choice
• A combination of CT scanning and contrast MRI is the imaging
regimen of choice for glomus jugulare tumors.
64

65. C T shows
• Soft tissue mass in the tympanic cavity
that may or may not erodes the jugular
foramen
• The ossicles may be displaced upwards.
• Destruction of floor of tympanic cavity.
65

66. • Enhance intensely with contrast
• Erosion of normal crest of bone between carotid
canal and jugular fossa-Phelps sign
• Moth eaten appearance due to destruction of
bony rim
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67. CT imaging demonstrates the extent of bony destruction (white
and black arrows) by the tumor. The normal jugular foramen on
the left (arrow head) is shown for comparison.
67

68. 68

69. Magnetic resonance imaging (MRI)
• Magnetic resonance imaging (MRI) with gadolinium-
diethylenetriamine pentaacetic acid (DTPA) contrast is best for
delineating tumor limits.
• T1-Weighted images-intermediate intensity
• T2-Weighted images-Hyperintense with strong contrast
enhancement.
69

70. • Glomus tumors on T1- and T2-weighted MRI have
characteristic soft tissue mixed intensity with
intermixed high-intensity signals and signal voids -
salt and pepper appearance representing fast
flowing blood
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71. T2-weighted axial MRI
• Salt and Pepper appearance
of the tumour resulting from
flow voids.
71

72. T1-weighted coronal slices
• It shows the involvement
of the jugular bulb and
the sigmoid-jugular
complex (arrows).
72

73. • Special subtraction MRI sequences may help distinguish
a glomus tympanicum tumor from a glomus jugulare
tumor.
73

74. Arteriography
• Unless carotid arteriography is necessary for preoperative
evaluation and/or embolization, noninvasive techniques are
preferred
• For large tumors involving the internal carotid artery (ICA),
preoperative carotid arteriography with cross-compression or
trial balloon occlusion is recommended.
74

75. Angiographic Imaging
• Arrows delineate the tumor
blush.
• The arrowhead
demonstrates a branch of
the middle meningeal artery
providing blood supply to the
tumor.
75

76. Common feeding vessels
• The main blood supply is via the ascending pharyngeal artery
from the external carotid artery (ECA) and branches from the
petrous portion of the internal carotid artery (ICA)
• Larger glomus jugulare tumors may also have blood supply
from other branches of the ECA, ICA, vertebral artery, and
thyrocervical trunk.
76

77. • After angiography, superselective embolization of the
tumor-feeding vessels is performed at the same time.
• Performed 1-2 days before excision
• Advantages- Decreased operative time
Decreased blood loss
Complete resection
77

78. • During cerebral angiography, the patency of the venous
drainage system of the opposite side and the patency of the
cerebral cross flow are evaluated.
• For tumors with large intracranial extension, vertebral
arteriography is advised to exclude arterial feeders from the
posterior circulation.
78

79. MAGNETIC RESONANCE ARTERIOGRAPHY AND
VENOGRAPHY
• Flow-sensitive weighting of the MRI can produce a magnetic
resonance arteriography and magnetic resonance venography
that are very useful in imaging glomus tympanicum lesions.
• The flow-sensitive modalities can indicate occlusion of the
jugular bulb and vein, which can be particularly helpful for
identifying involvement of the jugular bulb.
79

80. • A particular value of flow-weighted MRI is
that it can be a useful screening test for
synchronous paragangliomas.
• Provide information on relationship of
tumour to great vessels.
80

81. Retrograde venography
• The venous drainage systems also need to be carefully
studied before sinus occlusion is carried out during
surgical resection.
• It also help to differentiate between glomus jugulare
and glomus tympanicum.
• Glomus jugulare shows a filling defect.
81

82. OCTREOTIDE SCANNING
• I 123 Labelled Tyr 3 octreotide
• Paragangliomas have type 2 receptors on cell surface
for somatostatin analogues
• More sensitive(97%) and specific (82) for diagnosis
• Localisation < 1cm
• Detect recurrent lesion-Do not interfere with post
operative changes
82

83. PET SCAN
• Detect multiple site paraganglioma with SDHD and
SDHB gene mutation
• Patients with known metastatic disease
• Sensitivity-100%
83

84. GENETIC TESTING
• Recommended for patients with positive family history
or multifocal disease.
• Identification of mutation useful in –
Prognosis
Predicting risk of malignancy
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85. GLOMUS TUMOURS
Treatment
85

86. • Treatment is palliative or definitive (curative).
• Definitive treatment is surgical.
• RT is considered a palliative therapy.
86

87. • No lesion is technically unresectable.
• Each treatment plan is based on data generated by the
diagnostic evaluation
• Must consider patient factors such as age, tumor
type,natural history, and general medical health.
87

88. • Palliation is reserved for the elderly, medically infirm,or those
select, multicentric lesions in which definitive treatment is
otherwise contraindicated.
88

89. • Radiation therapy can be used alone; however, it is usually
reserved for patients with concurrent medical problems or the
elderly(65-75 years)who may be at higher risk for surgical
complications.
89

90. SURGICAL MANAGEMENT
• One of the most challenging procedures in head and
neck surgery.
90

91. BASIC PRINCIPLES
• Exposure of all tumor margins
• Identification/control of vital regional anatomy
• Access to all margins of Intracranial Extention
91

92. • During surgery in the region of the jugular foramen, cranial nerves
VII-XII are particularly vulnerable.
• Any damage to the seventh cranial nerve results in far more serious
morbidity than impairment of one or more of the other cranial
nerves.
• Continuous intraoperative facial nerve monitoring should be
performed routinely.
92

93. • The facial nerve nearly always causes a major obstruction to
the direct surgical access of the jugular foramen.
• Various surgical approaches have been described, all with the
intention of preserving the anatomical integrity of the facial
nerve in combination with adequate surgical exposure.
93

94. • The internal carotid artery is another major issue for
concern.
• The vertical part of the carotid canal lies close to venous and
nervous structures in the jugular foramen.
• Some lesions invade the petrous carotid artery, which makes
preservation of this vessel problematic.
94

95. Preoperative embolization
• If surgical removal is indicated, some tumours should be
previously embolized.
• Selective arteriography is performed 24 to 48 hours prior to
surgery.
• Tumour is embolized via branches of the external carotid
artery.
95

96. • Care is taken to embolize only those branches of the external
carotid artery that are feeders to the tumour, in order to avoid
neurological problems caused by reflux of embolic material
into the intracranial circulation.
• There is general agreement that preoperative embolization
significantly reduces blood loss during surgery and this
facilitates complete removal of the tumour.
96

97. • Excessive bleeding can lead to incomplete tumour
resection, damage to adjacent structures and post -
operative morbidity.
• It is therefore sensible to assess their vascularity by
angiography and undertake superselective embolization
in selected cases.
97

98. Relevant anatomy of the area
• The walls of the jugular foramen are formed anterolaterally by
the petrous bone and posteromedially by the occipital bone.
• The canal follows an anterior, inferior, and lateral direction to
exit the skull.
• Glomus tumours usually arise in the lateral part of the jugular
foramen
98

99. 99

100. • The posterolateral portion of the foramen (pars venosa)
contains the jugular bulb, posterior meningeal artery
• The anteromedial portion (pars nervosa) contains the inferior
petrosal sinus and cranial nerve IX,X and XI, Superior ganglion
of glossopharyngeal nerve and jugular ganglion of vagus nerve.
• The jugular bulb is situated between the sigmoid sinus and the
internal jugular vein.
100

101. 101

102. SURGICAL TECHNIQUE
102

103. Glomus Tympanicum
GLASSCOCK-JACKSON CLASSIFICATION
• Type I : Small tumor limited to Promontory.
Type II: Tumor completely filling Middle Ear Space.
Type III: Tumor filling middle ear & extending into Mastoid
process.
Type IV: Tumor filling middle ear, extending into mastoid or
through tympanic membrane to fill external auditory canal,
may extend anterior to internal carotid artery
103

104. • For a Class I tympanicum tumor - complete resection can be
accomplished by means of a transcanal tympanotomy.
• The mass is avulsed from the promontory, and bleeding is
controlled by microbipolar coagulation or light packing.
104

105. 105

106. • For a Class II - IV Glomus Tympanicum- a transmastoid
approach /Extended facial recess is elected.
106

107. Extended facial recess approach
• In radiologically proven glomus tympanicum tumors whose
borders are not entirely visible through the tympanic
membrane, the extended facial recess approach is
recommended.
107

108. • A cortical mastoidectomy is done.
• Facial nerve is identified and facial recess is opened via a
diamond burr.
• Chorda tympani is identified and transected.
• The annulus is used as a lateral land mark to hypotympanum.
108

109. • Total tumor ressection done after exposing
I C A and E T opening.
• Removal of retrofacial air cells provide complete exposure of
hypotympanum.
• After tumor ressection middle ear is packed with gel foam.
109

110. 110

111. • GLOMUS JUGULARE
Type I : Small tumor involving the jugular bulb, middle ear and
mastoid.
Type II: Tumor extending under the Internal Auditory Canal. There
may be intracranial extension.
Type III: Tumor extending into the Petrous Apex. There may be
intracranial extension.
Type IV: Tumor extending beyond the petrous apex into the clivus
and Infratemporal Fossa. There may be intracranial extension.
111

112. • Glomus Jugulare Tumor Class I and II (Small to Medium)-
Lateral Transtemporal surgical approach
112

113. Lateral Transtemporal surgical approach
• A wide retroauricular incision is made to gain access to the
lateral aspect of the temporal bone .
• This incision is extended downwards into the neck to expose
the structures in the infratemporal region.
113

114. 114

115. • Wide cortical atticomastoidectomy is performed with
skeletonization of the sigmoid sinus.
• A cranially based tympanomeatal flap can be
developed for exposure of the hypotympanic region.
115

116. • After detachment of the tendon of the
sternocleidomastoid muscle, the mastoid tip is
removed.
• The mastoid segment of the fallopian canal is
skeletonized, from the second genu towards the
stylomastoid foramen.
116

117. • The bone anterior to this part of the facial nerve is
removed and, at the same time, the dome of the jugular
bulb is exposed.
• A diamond burr is used to remove as much bone as
possible that covers the sigmoid sinus and the jugular
bulb, so that the Fallopian bridge is the only structure
that overlies the jugular bulb.
117

118. 118

119. 119

120. • As a result, the facial nerve may impair adequate
exposure of lesions involving the jugular foramen, but
the degree of access required also depends on the
pathology
• In many cases, the vertical segment of the facial nerve
has to be transposed, particularly if the lesions are
extensive and bulky.
120

121. • The different approaches to facial nerve are as
follows
121

122. CONSERVATIVE FACIAL NERVE MANAGEMENT
• Anatomical relationship between the mastoid segment of the
facial nerve and the jugular bulb varies considerably.
• When the space between the two is small, it may be difficult to
achieve adequate exposure.
122

123. • After the atticomastoidectomy, the sigmoid-jugular
complex is exposed.
• The facial nerve is skeletonized and left in situ.
• The sigmoid-jugular complex is controlled by sutures
around the sigmoid sinus and the internal jugular vein.
123

124. The right jugular foramen region is exposed without
transposition of the facial nerve.
• 1, External auditory canal; 2, parotid
gland;
• 3, glossopharyngeal nerve; 4,
hypoglossal nerve;
• 5, internal carotid artery;
• 6, vagus nerve;
• 7, internal jugular vein;
• 8, spinal accessory nerve; 9, jugular
bulb;
• 10, facial nerve;
• 11, sigmoid sinus;
• 12, incus;
• 13, semicircular canals.
124

125. • Some type of facial nerve transposition may need to be
considered if visibility is poor and there is always the
risk of incomplete tumour removal.
125

126. FACIAL NERVE TRANSPOSITION EAR, CANAL WALL
UP
• This technique involves complete removal of the bony covering
of the mastoid segment of the facial nerve, from the second
genu down to the stylomastoid foramen.
• Outside the stylomastoid foramen, the nerve is followed and
mobilized over its intraparotid course: first the main trunk and
then the temporozygomatic and cervicofacial branches.
126

127. • The subdivisions of these branches are also mobilized
and the overlying parotid tissue is removed.
• After this part of the procedure there is some mobility
of the nerve, such that it can be pulled anteriorly, away
from the jugular bulb.
127

128. The right jugular foramen region is exposed with a limited
transposition of the facial nerve.
• After an atticomastoidectomy, the
mastoid segment of the facial nerve
is uncovered.
• The intraparotid part of the nerve
is exposed and mobilized.
• The nerve can then be pulled
anteriorly in order to provide access
to the jugular bulb
128

129. FACIAL NERVE TRANSPOSITION EAR, CANAL WALL
DOWN
• In order to be able to mobilize the facial nerve more
extensively, atticomastoidectomy should be extended to
subtotal petrosectomy.
• After a canal wall down atticomastoidectomy, the tympanic
membrane, the malleus and the incus are removed.
129

130. • As a result, the facial nerve can be uncovered, not only in its
mastoid segment, but also in its tympanic part.
• After mobilization of the intraparotid facial nerve, the nerve
can be rerouted anteriorly.
• The hinge point (pivot) may be located at the second genu, but
wider exposure is achieved when the nerve is displaced
anteriorly with the hinge point at the first genu.
130

131. Facial nerve transposition canal wall down.
• The right jugular foramen region is
exposed using a more extensive
transposition of the 7th nerve.
• The facial nerve is uncovered in its
mastoid and tympanic segments.
• The intraparotid part of the nerve is
exposed and mobilized.
131

132. Facial nerve transposition, canal wall down.
• Maximum exposure of the
right jugular foramen is
achieved by rerouteing the
facial nerve anteriorly with
the hinge point at the first
genu
132

133. MANAGEMENT OF THE SIGMOID-JUGULAR
COMPLEX
• Damage to the sigmoid sinus or the jugular bulb is a serious
risk during jugular foramen surgery.
• If the vessel wall is punctured, the initial bleeding is fairly
simple to control, but retrograde haemorrhage from the
internal jugular vein may follow.
133

134. • Therefore, the venous blood flow must be blocked at two
levels: cranially and caudally to the lesion, i.e. upstream and
downstream
• It is logical to start with blocking the main flow on the cranial
side, the sigmoid sinus.
• Two methods have been described: ligating the vessel or
packing it with Surgicel
134

135. 135

136. • The upper neck can be explored by extending the
retroauricular incision downwards.
• Internal jugular vein can be found easily in a later stage of the
procedure, after the sternocleidomastoid muscle has been
detached from the mastoid process and the mastoid tip has
been removed.
136

137. • After identifying the surrounding structures, such as the
internal carotid artery and the cranial nerves IX, X, XI and XII,
the internal jugular vein should be ligated as high up as
possible to avoid bleeding from veins that drain into it in the
upper neck.
137

138. • The inferior and superior petrosal sinuses and the mastoid and
condylar emissary veins drain into the sigmoid-jugular
complex.
• Usually in a later stage of the procedure, the sigmoid sinus and
the jugular bulb have to be opened to remove the tumour.
• Then bleeding can be expected from these sinuses and
emissary veins, which should be dealt with by firm packing
with Surgicel.
138

139. MANAGEMENT OF THE INTERNAL CAROTID ARTERY
• Damage to the internal carotid artery is likely to be
followed by uncontrollable arterial haemorrhage.
• The tumour may be attached to the internal carotid
artery or be in close contact with it.
139

140. • Visualization of the plane between the tumour and the petrous
carotid artery is extremely important.
• Operative field should be as bloodless as possible.
• All sources of venous haemorrhage must be meticulously
controlled, either by ligation or by packing with Surgicel.
140

141. • Glomus tumours may invade the carotid canal, first in its
vertical part and eventually along the horizontal part on
to the foramen lacerum.
• Glomus tumours may also receive part of their blood
supply from the petrous carotid artery, e.g. via its
caroticotympanic or dural branches.
141

142. • In very large tumours, permanent balloon occlusion of
the internal carotid artery must be considered, but only
if there is sufficient collateral circulation.
• It can be tested preoperatively during the angiography
procedure by temporary occlusion.
142

143. • Extensive lesions and malignant tumours may require
wide resection with sacrifice of the internal carotid
artery.
• In these cases an extracranial-intracranial bypass
procedure has to be considered
143

144. • Tulleken et al. described an extracranial-intracranial
bypass technique that is considerably safer, because it
precludes the ligation of intracranial vessels.
• In this method, first a venous graft or transplant is
connected to the bifurcation of the carotid artery in the
neck
144

145. • This transplant is connected end-to-side to the recipient
posterior cerebral artery.
• Relatively safe alternative to permanent balloon occlusion.
145

146. (a) A platinum ring is attached to the outside of the recipient
artery, at the bifurcation of the intracranial internal carotid
artery.
146

147. (b) The end of a venous transplant is attached to the recipient
artery and sutures around the platinum ring fix the recipient
artery to the donor.
147

148. • (c) A specially designed
catheter with the excimer
laser is introduced in the
venous transplant.
148

149. • (d) The tip of the laser touches the wall
of the artery.
• The platinum ring ensures proper
positioning of the laser tip.
• This device has a circular configuration.
• Laser fibres penetrate the recipient
artery after evaporization of the vessel
wall.
149

150. • (e) The catheter is withdrawn along with a
full-thickness portion of the recipient
artery wall.
• A temporary clip is applied to the
transplant.
• A conventional end-to-side anastomosis is
made between the other end of the
venous transplant and the external carotid
artery in the neck.
• The bypass is functional after removal of
the temporary clip.
150

151. • The sigmoid sinus and jugular bulb are opened to remove the
tumour.
• After tumour resection, the cavity is filled with abdominal fat
and covered with a pedicled temporalis muscle flap.
• Fibrin glue is also used routinely to prevent cerebrospinal fluid
leakage.
151

152. • Lumbar drainage may be considered if the resection of a
bulky tumour has left a voluminous cavity with wide
access to the posterior fossa.
• Blind sac closure of the external ear canal is performed
according to current standards.
152

153. • Glomus Jugulare Tumor Class III and IV (Medium to Large)-
infratemporal fossa approach
153

154. The infratemporal fossa Approach
• Fisch (1979) has excised lesions of the skull base and petrous
apex previously considered unresectable through the
infratemporal fossa approach.
• The infratemporal fossa approach of Fisch (Fisch, 1977; Fisch
and Pillsbury, 1979; Fisch et al, 1984) encompasses three
distinct variations for use in specific clinical situations
154

155. • The type A approach provides exposure between the
sigmoid sinus and the condylar fossa, designed to reach
to the petrous apex and infralabyrinthine areas.
• It is most useful for management of cholesteatomas,
meningiomas, and glomus tumors of those regions.
155

156. • The type B approach allows access from the sigmoid
sinus to the petrous tip (including exposure of the
horizontal ICA and foramen ovale) to reach lesions of
the clivus, such as chordomas, meningiomas, glomus
tumors and extensive apex cholesteatomas.
156

157. • The type C approach expands this access to include the
parasellar region, the cavernous sinus, foramen rotundum, and
foramen lacerum.
• Removal of the pterygoid plates in this approach also
facilitates access to the nasopharynx.
• This type C approach is used in the resection of small
nasopharyngeal carcinomas, adenoid cystic carcinomas, and
angiofibromas
157

158. Procedure
• All three variations of the infratemporal fossa approach
involve mastoidectomy, facial nerve dissection (and
transposition), and obliteration of the eustachian tube,
middle ear, and external auditory canal with resultant
permanent conductive hearing deficit.
158

159. • It requires identification in the neck of the cranial
nerves of the jugular foramen, transposition of the
facial nerve, and removal of all the bone lateral to the
tumor and jugular bulb.
• A wide mastoidectomy is performed, and the facial
nerve is identified from the cochleariform process into
the parotid gland and transposed anteriorly.
159

161. • The sigmoid sinus is packed off and opened, and the
jugular vein is ligated and divided in the neck.
• The jugular vein is then elevated superiorly up to the
jugular bulb, with care being taken to visualize and
preserve the cranial nerves on its anteromedial surface.
161

162. • The tumor mass is then dissected free working the tumor
margin in a circumferential fashion.
• As the tumor bleeds it is packed off with Surgicel, and a new
area is dissected.
• Anteriorly, care must be taken to avoid injury to the carotid
artery.
• Identification of the carotid artery remote from the tumor may
be helpful in the development of this plane.
162

163. • Resection of posterior fossa dura, facial nerve, cochlea,
and semicircular canals may be required in larger
tumors.
• Any dural defects that exist are repaired.
• The surgical defect is packed with an abdominal fat
graft.
163

164. • A subtotal petrosectomy anterior to the labyrinth exposes the
entire intrapetrous course of the ICA.
• The facial nerve is transposed anteriorly for improved access.
• The petrous apex may be obliterated, the eustachian tube
packed, and the external auditory canal closed laterally.
164

168. • The external auditory canal skin may be oversewn after
removal of the deep external auditory canal skin.
• A conductive hearing loss, therefore, results.
• This approach has been reported in cases involving glomus
tumors extending to the petrous apex.
168

169. Postoperative Details
• Careful monitoring of cardiac function is advisable-if a
catecholamine secreting tumor was only partially resected.
• Postoperative lower cranial nerve deficits need to be carefully
diagnosed -early rehabilitation is advocated
169

170. Complications of surgery:
• Death, cranial nerve palsies, bleeding, wound infection,
cerebrospinal fluid (CSF) leak, meningitis, Stroke,
Aspiration Pneumonia, uncontrollable
hypotension/hypertension, Hearing loss and tumor
regrowth.
170

171. NON SURGICAL MANAGEMENT
171

172. Wait and see policy
• In the pre-CT and MRI era, just the presence of a space-
occupying lesion used to form sufficient grounds to proceed
directly to surgery.
• Now a days, the size of a jugular foramen lesion can be
accurately measured, monitored and documented with the
available imaging modalities of CT and MRI.
172

173. • Most common lesions in the jugular foramen are frequently
either slowly progressive or in a steady state.
• In many cases, the lesions do not pose a threat during the
whole of the patient’s life.
173

174. • Slow growth and benign nature of majority of paragangliomas
watchful waiting or observational may be considered for
selected patients
• Elderly patients, Small tumours, Multiple comorbidities
• Follow up imaging after 6 months and 12 months thereafter.
174

175. Radiotherapy
• There is no consensus about whether conventional
fractionated radiotherapy is beneficial to patients with glomus
tumours, because these tumours are histologically benign.
• The aim of radiotherapy for glomus tumours
to destroy the microvascular tissue and bring about sclerosis
and fibrosis.
175

176. • Dose - 2000 to 3000 rads
• Advantage – pre op radiotherapy shrinks the tumor , decreases
its vascularity.
• Disadvantage-damaging the surrounding structures, such as
the cochlea, the facial nerve and adjacent brain tissue & cause
radionecrosis of temporal bone.
176

177. • Radiotherapy for glomus tumours may also be an option
in patients who refuse surgery, are elderly or have a
poor physical condition.
177

178. Stereotactic radiosurgery
• Stereotactic radiosurgery is rapidly gaining popularity as
a treatment modalIty.
• The term 'gamma knife stereotactic radiosurgery'
describes the major components of the technique.
178

179. Systemic Therapy
• Malignant paraganglioma-palliative therapy
• Reduce or stop the rate of growth
• Combination of cyclophosphamide, vincristine, Dacabazine
and radionucleotide Iodine13I MIBG therapy
• 5 year survival < 50%
179

180. Investigational Therapy
• Radiotherapy therapy with radiolabelled somatostatin
analogues
• Sunitinib- Oral tyrosine kinase inhibitor prevent angiogenesis
180

181. Recurrence
• Glomus Tympanicum- <1%
• Glomus Jugulare-5-10%
181

182. Follow-up
• GTy tumors yearly for 5 years and once every 5 years
thereafter.
• Glomus jugulare-Because of their propensity for late
recurrence and multicentricity,
postoperative MRI surveillance should be conducted 1year, 3
years, 5 years, and then every 5 years postoperatively for the
life of the patient
182

183. THANK YOU
183

184. THANK
YOU