Glomus tumours are benign, slow-growing, hypervascular tumours that originate from glomus bodies in the middle ear. They are most commonly seen in the 5th decade of life and affect females more often than males. On histology, they appear as clusters of cells arranged in a nested pattern surrounded by a vascular stroma. Surgical excision is the primary treatment, though pre-operative embolization of feeding vessels can help reduce blood loss. Glomus tumours can spread locally within the temporal bone and occasionally metastasize to distant sites like the lungs. Advanced cases may require a combined surgical and radiotherapy approach.

1. GLOMUS TUMOUR
DR UTKAL MISHRA
VSS MEDICAL COLLEGE, BURLA

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 derived from neural crest.

3. SYNONYM
 Chemodectoma
 Paraganglioma
 Ganglia Tympanica
 Vascular tumour of middle ear

4. HISTORY
 1840 – Valentine described it first as Ganglia Tympanica.
 1902 – Guild found similarities between these tumour & carotid body & coined the term Glomus
Jugulare.
 1924 – Mason was first to describe Glomus tumours as hyperplastic glomus bodies.
 1945 – Rosenwassser was first to diagnose a patient with glomus tumour & it’s surgical excision.

5. WHAT ARE PARAGANGLIA ???
 Paraganglia cells are derived from the neural crest.
 Histologically, they resemble carotid body.
 In middle ear paraganglia are distributed over –
1. Promontory – Along the branches of tympanic branch of glossopharyngeal N. or auricular br. Of vagus
2. Dome of jugular bulb – Adventitial layer
 Paraganglia contain two types of cells:
Type 1 → Chief cells or Granular cells → Release catecholamine
Type 2 → Supporting or Sustentacular cells.

6. INCIDENCE
 1 in 100000
 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.
 Commonly affected ear - LEFT

7. PATHOPHYSIOLOGY
 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.
 Intially erodes in region of jugular fossa & posteroinferior petrous bone with subsequent
extension to the mastoid & adjacent occipital bone.
 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.

8. HISTOLOGY
 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.
 Guild classified Glomus tumours into 2 types histologically –
1. Cellular Glomus
2. Vascular Glomus

9. TYPES
 2 types according to site of origin –
1. GLOMUS TYMPANICUM – Arising from promontory.
2. GLOMUS JUGULARE – Arising from dome of jugular bulb.

10. RULE OF 10
 10 % Multicentric
 10 % Familial
 10 % Functional

11. CLINICAL FEATURES
When tumor is Intratympanic –
 1. Earliest symptoms are deafness (conductive) and pulsatile tinnitus abolished by carotid pressure.
 2. Otoscopy - Red reflex, Rising Sun appearance, Bulging TM.
 3. Browne’s Sign - When ear canal pressure is raised with Siegel's speculum, tumor pulsates vigorously
and then blanches
 4. Aquino sign - It is blanching of the mass with manual compression of ipsilateral carotid artery.

12. CLINICAL FEATURES
When tumor present as polyp -
 1. History of profuse bleeding from the ear either spontaneously or on attempts to
clear it.
 2. Dizziness, vertigo, Facial Paralysis, earache, otorrhea.
 3. Audible bruit : Heard by stethoscope over mastoid at all stages.

13. CLINICAL FEATURES
 Multiple Cranial Nerve Palsies IX, X, XI, XII
 Late feature apppearing several years after ear symptoms
 Dysphagia, Hoarsenes, Palatal Palsy
 Atrophy of tongue muscles
 Weakness of Trapezius & Sternocleidomastoid M.

14. CLASSIFICATION
 LUNDGREN CLASSIFICATION
 GLASSCOCK- JACKSON CLASSIFICATION
 FISCH CLASSIFICATION
 GUILD HISTOLOGICAL CLASSIFICATION
 MODIFIED DE LA CRUZ CLASSIFICATION

15. LUNDGREN CLASSIFICATION
 Glomus Tympanicum
 Glomus Jugulare

16. 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

17. GLASSCOCK - JACKSON CLASSIFICATION
 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.

18. FISCH 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
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 canal
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

19. DIFFERENTIAL DIAGNOSIS
 Otitis Media
 Otosclerosis
 Cholesterol Granuloma
 Aberrant Intrapetrous Internal Carotid Artery
 Idiopathic Hemotympanum
 Aneurysm
 Arteriovenous Malformation
 Prominent jugular bulb
 Persistent stapedial artery

20. SPREAD OF GLOMUS TUMOUR
 Perforate TM → Polyp
 Invade Mastoid, Labyrinth & Petrous pyramid
 Invade Jugular foramen & Base of skull → IX to XII Cr N. Palsy
 Eustachian Tube → Nasopharynx
 Spread Intracranially to Posterior & Middle cranial fossa
 Metastasis to lungs & bones (Rare)

21. INVESTIGATION
 CT Scan of head with Bony window 1mm slice → PHELP’S SIGN –
Obliteration of CJ spine
 Gd enhanced MRI – Multiple vascular flow voids→ Salt And Pepper
Pattern
 4 vessel Angiography – Commonest feeding vessel → Inferior
Tympanic Br. Of Ascending Pharyngeal A.
 Radionuclide Scintigraphy – To detect multifocal Para-ganglioma
 Serum catecholamine levels.
 24 hr urine for catecholamine , metanephrine & VMA.

22. TREATMENT
 Treatment of choice – Microsurgical total tumor removal after pre – op embolization of feeding
vessel.
 Inoperable case - Radiation

23. ROLE OF RADIOTHERAPY
 Controversial
 Fibrosis of arterioles rather then direct affect on tumour cells.
 Stereotactic radiotherapy , achieve tumour control rate of 80 – 90 %.
 Contraindication – Intracranial Extention

24. SURGICAL APPROACHES
 Glomus Tympanicum with entire circumference visible - Transcanal approach
 Glomus Tympanicum with extension to hypotympanum - Hypotympanic approach
 Glomus Tympanicum extending to mastoid - Extended Facial Recess approach
 Glomus Jugulare not extending to ICA, Neck, Postr. Fossa - Mastoid Neck approach
 Large Glomus Jugulare - Infratemporal fossa approach
 Tumours extending towards Foramen Magnum - Transcondylar approach

25. MODIFIED DE LA CRUZ CLASSIFICATION

26. TRANSCANAL APPROACH

27. EXTENDED FACIAL RECESS APPROACH

28. MASTOID NECK APPROACH

29. MASTOID NECK APPROACH

30. MASTOID NECK APPROACH

31. MASTOID NECK APPROACH

32. PRESENT SCENARIO
 There is no MRI
 No Interventional Radiologist
 No Operating Microscope
 No Light Source !!!!
 Only management → URGENT REFERRAL

33. THANK
YOU