Glomus tumors are rare, slow-growing, hypervascular tumors that originate from glomus bodies. They most commonly occur in the middle ear (glomus tympanicum), jugular bulb region (glomus jugulare), or vagus nerve in the neck (glomus vagale). Surgical resection is the primary treatment, with the surgical approach depending on the size and extent of the tumor. While benign, glomus tumors can cause cranial nerve deficits if they invade local structures and have a small malignant potential. Long-term monitoring is important to check for recurrence.

2.  Glomus tumour also known as chemodectoma / non
chromaffin paragangloima/ fibroangioma is a rare
slow growing , hypervascular tumour.
GLOMUS BODIES – described by Guild in 1941 as
“small highly vascular mass of epitheloid cells
located in the region of adventitia of the jugular
bulb” .
 Valentine in 1840 first described it as ganglia
tympanica.
 Misnomer.

3.  Paraganglions – clusters of chief cells supported by
sustentacular cells and small blood vessels.
Distibuted along the course of autonomic nerves
in skull base , thorax & neck.
Chemoreceptor tissues.
Derived – cells of neural crest.
Compensatory physiological response
to hypoxia , hypercapnia & acidity.
Secrete – catecholamines &
neuropeptides

5.  Largest concentration of these paraganglion cells –
adrenal medulla.
 Temporal bone - adventitia of jugular bulb.
tympanic canaliculus.
retrofacial air cells.
promontory & geniculate ganglion.
Neck – vagal body , carotid body , along laryngeal nerve
& in the mediastinum.
Rosenwasser first reported origin of tumour glomus
juglare in 1945.

7.  Most common bening tumour of middel ear.
 2nd most common tumour of cerebropontine angle &
temporal bone.
 Estimated annual incidence – 1/ 1.3 million ppl.
 5th & 6th decade of life.
 Male to female ratio 4-6: 1.
 No ethnic or racial prediliction.
 2 to 4 % malignant.
 5 % secrete neuroactive peptide ( functional ).
 10 % multicentric.
 MEN 2A & 2B.
 10 % familial - autosomal dominant ( gene 11q23).

8.  Encapsulated , slowly growing tumour , highly vascular
and locally invasive.
 Expand within temporal bone via pathways of least
resistance – air cells , vascular lumens , skull Base
foramina & the eustachian tube.
 Invade & erode bone in lobular fashion.
 Intially erodes in region of jugular fossa &
posteroinferior petrous bone with subsequent
extension to the mastoid & adjacent occipital bone.
 Intracranial & extracranial extension occur.
 Metastasis – lung , lymph node , liver , spleen.

9.  Glomus tympanicum
– confined to middle
ear space.
 Glomus jugulare –
arise in region of
jugular
 Glomus vagale – arise
in neck , extending
towards jugular
foramen.
 Carotid body tumour
– area of carotid
bifurcation.

10.  Most of jugulotympanic paraganglia located in the
adventitia of jugular bulb.
 Blood supply – ascending pharyngeal artery &
branches from petrous portion of ICA.

11.  Jugular foramen :- walls are formed anterolaterally by
petrous bone.
 Posteromedially by the occipital bone.
 Canal – anterior , inferior & lateral direction to exit the
skull.
 Posterolateral portion( pars venosa) – jugular bulb ,
posterior meningeal artery & cranial nerve X & XI.
 Anteromedial portion ( pars nervosa) – inferior petrosal
sinus & IX.
 Jugular bulb situated between sigmoid sinus & IJV.
 Lower cranial nerve are situated medial to medial wall of
jugular bulb .
 IAC , PSCC, middle ear , EAC, facial nerve & ICA within
the carotid canal.
 Extracranial end - ICA , IJV , nerves VII, X , XI , XII within 2cm
area.

14. Glomus tympanicum.
 I – small Tm confined to
promontary.
 II – Tm completely filling
middle ear space.
 III – Tm filling middle ear
& extending into mastoid
process.
 IV – Tm filling middle ear ,
extending into mastoid
process or through TM to
fill EAC , may extend
anterior to IAC.
 I – Tm involving jugular
bulb , middle ear &
mastoid process.
 II – Tm extending under
IAC ; may have intracranial
extension.
 III – Tm extending into
petrous apex ; may have
intracranial extension.
 IV – Tm extending beyond
petrous apex into clivus or
infratemporal fossa ; may
have intracranial
extension.
Glomus jugulare.

15.  Type A – Tm localized to middle ear cleft.
Type B – Tympanomastoid Tm with no destruction of bone
in the infralabrynthine compartment of the temporal bone.
Type C – Tm invading the bone of the infralabrynthine
compartment of temporal bone.
C1 – Tm with limited involvement of vertical portion of
carotid canal.
C2- Tm invading vertical portion.
C3- Tm invading the horizontal portion.
Type D – Tm with intracranial extension.

16.  Type A Type B

17. Type C1 Type C2

18. Type C3 Type D

19.  CLINICAL PRESENTATION
Tm of slow growth with average delay of 6 years.
 When Tm is intratympanic – earliest & most
common symptom is deafness & pulsatile tinnitus
(swishing character , synchronous with pulse ).
Deafness – mainly conductive & mixed.
 When Tm present as polyp – h/o profuse bleeding.
Earache( uncommon) , aural fullness.
Otorrhoea – 2nd infection with polyp.
Dizziness , vertigo & facial palsy.

20.  Cranial nerve palsies – Tm invade jugular foramen
& base of skull. late feature appear several years
after aural symptoms.
 Cranial nerve IX- XI with or without XII.( jugular
foramen / Vernet’s syndrome).
 Dysphagia & hoarseness with u/l paralysis of soft
palate , loss of gag reflex , parotid gland secretions
with weakness of trapezius & scm muscle.

21.  May present as mass over mastoid or through ET
as mass in nasopharynx.
 Intracranial extension – headache , hydrocephalus
& raised ICT.
 Metastasis – lymph node mass.
 Functional Tm - headache , palpitations ,
tachycardia, sweating , hypertension & anxiety.

22.  Otoscopic examination – characterstic , pulsatile ,
reddish – blue mass behind the TM .
 Rising sun appearance –
Tm arise from
floor of middle ear cavity.
 Polyp can be seen.
 Brown’s sign – when EAC pressure is raised with
siegle’s speculum Tm pulsates vigourously & then
blanches ; reverse happens with release of
pressure.

23.  Aquino sign – blanching of mass with ipsilateral
carotid artery compression.
 Cranial nerve palsies.
 Auscultation – systolic bruit over mastoid.
 Audiological examination – conductive & or mixed
hearing loss.
 Other features according to extension

24.  Determination of Tm size , type & extent.
 Evaluation of histochemical or multicentric associated
lesion.
 Identification & assessment of ICE.
 Assessment of major vasculature involvement.
 Assessment of intracranial collateral circulation.

25.  H/o & physical examination.
 Routine investigations.
 Audiological investigation.
 Serum catecholamine levels.
 24 hr urine for catecholamine , metanephrine &
VMA.

26.  Mainstay of diagnosis is radiological imaging.
 CT scan
 MRI
 Angiography.
 Radionuclide scintigraphy.
 CT scan – Both axial & coronal plane
extent of lesion
bony erosion.
Caroticojugular plate.
Phelp’s sign

29.  MRI with gadolinium enhancement – Tm extent
Intracranial extension
Relationship of Tm to neural & vascular
structures .
Salt and pepper pattern ( vascular flow voids).
Angiography – vascular supply of Tm .
Evaluating ICA involvement.
Preop embolization of Tm.
Large Tm involving ICA , cross
compression or trial of balloon
occlusion is recommended.

32.  Definitive or palliative.
 Surgery , irradiation , embolization &
chemotherapy.
 Definitive T/t is surgery.
 Palliative – elderly , medically unfit or those with
multicentric lesion, possible postoperative
weakness of vagus or hypoglossal , or both nerves.
 Asymptomatic patients – GT observed with serial
imaging.

33.  Relative effectiveness is controversial.
 Primarily, palliatively & as surgical salvage.
 Fibrosis of arterioles rather then direct affect on Tm cells.
 Tm shrinks , bleeding ceases. Tinnitus & vertigo
improve.
Deafness & cranial nerve palsy persist.
Radiotherapy with or without intensity modification
Stereotactic radiotherapy , achieve Tm control rate of
80 – 90 %.
Contraindicated – ICE & osteomyelitis.
S/E – ICA thrombosis, CSF leak , necrosis of bone, brain &
dura , pitutary – hypothalamic insufficency.

34.  Aim is complete resection with minimal neurological
deficit.
 Type A – Glomus tympanicus approached via
transmeatal route.
 Type B – Combined approach ( extended facial recess )
 Type C – Skull base approach utilizing upper cervical
dissection and transmastoid approach.
 Type D – skull base approach with posterior fossa
craniotomy.

35.  Reduce Tm vascularity
Pre op irradiation.
Pre op embolization.

36.  CLASSIFICATION SURGICAL APPROACH
 Tympanic Transcanal
 Tympanomastoid Mastoid/extended facial re cess
 Jugular bulb mastoid/neck (facial rerouting)
 Carotid artery infratemporal fossa
 Transdural infratemporal fossa /
intracranial
 Craniocervical transcondylar
 Vagale cervical

37. If the margins of the lesion are visible around the entire
circumference of the lesion.
Middle ear is exposed via tympanomeatal flap.
Malleus may be seperated from the TM to gain
additional exposure.
Limits of Tm confirmed.
Microbipolar cautery is applied to the feeding
vessels & margins , and Tm is avulsed.
Tm is seperated from its attachement to promontary
mucosa.
Nerve of tympanic plexus is also involved.

39. Tympanomastoid Glomus Tm.
 Postauricular incision made & complete
mastoidectomy done.
 Facial recess is opened.
 Further bone is removed inferiorly with sacrifice of
chorda tympani.
 Expose the middle ear & hypotympanum.

40.  Tm may be removed from ossicular chain , facial nerve
& IJV.
 Using bipolar cautery shrinking of Tm & controlling
the blood supply.
 TM may be removed if it is involved.
 If necessary posterior wall of EAC is removed.

43.  Tm involving jugular bulb but doesn’t extend into
ICA or neck or PCF.
 Same exposure as extended facial recess approach.
 Additional amputation of mastoid tip done.
 Periosteum of diagastric groove is followed
anteriorly till it turns abruptly at stylomastoid
foramen.
 Drilling is done laterally along the ridge.
 Free the mastoid tip.
 Tip is removed.
 Incision is extended along anterior border of SCM.

44.  Posterior belly of diagastric muscle is identified.
 Freed from diagastric groove.
 Retracted anteriorly .
 Neurovascualr structures are exposed.
 IJV is identified & ligated.
 Jugular vein followed over the transverse process of
1st cervical vertebra into base of skull.
 Cranial nerve XI (lateral to vein ) identified.
 IX , X identified & preserved.
 Expose SS & JB.

45.  Proximal SS controlled with extraluminal packing of
surgicell.
 SS may be opened distal to the proximal packing.
 Tm is resected.
 Tm & ossicles if involved should be removed.

53.  Periosteum of the facial nerve at the stylomastoid
foramen is preseved.
 Fibrous attachement of nerve in its vertical part is
transected.
 Nerve is transported with the periosteum at the
attached belly of diagastric muscle.
 Less chances of injury to nerve.

54.  Transdural extension of the Tm.

55.  Bleeding.
 Uncontrolled hyper/ hypotension
 Cranial nerve palsises.
 CSF leak.
 Meningitis.
 Hearing loss.
 TM perforation.
 EAC stenosis.
 Tumour regrowth.
 Death.

56.  Radiological & when indicated endocrinological
monitoring of tumour regrowth is indicatedevery 6
months to 1 year for 2 year , then depending upon the
findings every 2 year.

57.  Glomus jugulare Tm grow slowly & may lead to cranial
nerve palsy.
 Which is benign .
 However , recent studies have shown long term
reduced quality of life.
 Mortality rate is6.2 % in patients treated with
radiation.
 2.5 % in surgically treated patients.
 Overall mortality is 8.7%.

58.  Surgery is the treatment of choice.
 Effectiveness will improve with intraoperative imaging
& guiding system.
 Recent genetic research on familial glomus tumour
suggest future direction of tumour towards gene
manipulation.