Glomus Tumour

Ent Postgraduate Seminar slide

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

2. INTRODUCTION
It is the commonest benign tumourof middle ear.
It is BENIGN, SLOW GROWING , HYPERVASCULAR tumour.
It is so named because of its origin from glomusbodies found over jugular bulb &
promontory.
It also contains paraganglioniccells derived from neural crest.

3. SYNONYM
Chemodectoma
Paraganglioma
Ganglia Tympanica
Vascular tumourof 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 Glomustumoursas hyperplasticglomusbodies.
1945 –Rosenwassserwas first to diagnose a patient with glomustumour& it’s surgical excision.

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

6. INCIDENCE
1 in 100000
5 times more common in female.
AutosomalDominant inheritance.
Gene responsible is located on chromosome –11q23
Age –Most commonly seen in 5
th
decade of life.
Commonly affected ear -LEFT

7. PATHOPHYSIOLOGY
Benign, Encapsulated, Slow growing, Highly vascular, Locally invasive tumourthat
erodes bone.
Expand within temporal bone via pathways of least resistance –air cells , vascular
lumens , skull Base foramina & the eustachiantube.
Intiallyerodes in region of jugular fossa& posteroinferiorpetrousbone with subsequent
extension to the mastoid & adjacent occipital bone.
The middle ear ossiclesare commonly spared.
Intracranial & extracranialextension occur.
Metastases from glomustumorsoccur 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 ZELLBALLENenclosed by
fibrous stromawith rich vascular plexus.
Guild classified Glomustumoursinto 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 tumoris Intratympanic–
1. Earliest symptoms are deafness (conductive) and pulsatiletinnitus 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, tumorpulsates vigorously
and then blanches
4. Aquino sign -It is blanching of the mass with manual compression of ipsilateralcarotid artery.

12. CLINICAL FEATURES
When tumorpresent 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 apppearingseveral years after ear symptoms
Dysphagia, Hoarsenes, Palatal Palsy
Atrophy of tongue muscles
Weakness of Trapezius& SternocleidomastoidM.

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

15. LUNDGREN CLASSIFICATION
GlomusTympanicum
GlomusJugulare

16. GLASSCOCK -JACKSON CLASSIFICATION
GLOMUS TYMPANICUM :
Type I : Small tumorlimited to Promontory.
Type II: Tumorcompletely filling Middle Ear Space.
Type III: Tumorfilling middle ear & extending into Mastoidprocess.
Type IV: Tumorfilling 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 tumorinvolving the jugular bulb, middle ear and mastoid.
Type II: Tumorextending under the Internal Auditory Canal.There may be intracranial extension.
Type III: Tumorextending into the PetrousApex.There may be intracranial extension.
Type IV: Tumorextending beyond the petrousapex into the clivusand InfratemporalFossa.There may
be intracranial extension.

18. FISCH CLASSIFICATION
Type A -Tumorlimited to Middle Ear (carries the best prognosis)
Type B -Tumorlimited to the TympanomastoidArea with no infralabyrinthinecompartment involvement
Type C -Tumorinvolving the InfralabyrinthineCompartmentof temporal bone with extension to petrousapex
Type C1 -Tumorwith limited involvement of the vertical portion of the carotid canal
Type C2 -Tumorinvading the vertical portion of the carotid canal
Type C3 -Tumorinvasion of the horizontal portion of the carotid canal
Type D -Tumorwith Intracranial Extension
Type D1 -Tumorwith an intracranial extension less than 2 cm in diameter
Type D2 -Tumorwith an intracranial extension greater than 2 cm in diameter

19. DIFFERENTIAL DIAGNOSIS
OtitisMedia
Otosclerosis
Cholesterol Granuloma
Aberrant IntrapetrousInternal Carotid Artery
Idiopathic Hemotympanum
Aneurysm
ArteriovenousMalformation
Prominent jugular bulb
Persistent stapedialartery

20. SPREAD OF GLOMUS TUMOUR
Perforate TM → Polyp
Invade Mastoid, Labyrinth & Petrouspyramid
Invade Jugular foramen & Base of skull → IX to XII Cr N. Palsy
Eustachian Tube → Nasopharynx
Spread Intracraniallyto 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
Gdenhanced 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 embolizationof feeding
vessel.
Inoperable case -Radiation

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

24. SURGICAL APPROACHES
GlomusTympanicumwith entire circumference visible -Transcanalapproach
GlomusTympanicumwith extension to hypotympanum-Hypotympanicapproach
GlomusTympanicumextending to mastoid -Extended Facial Recess approach
GlomusJugularenot extending to ICA, Neck, Postr. Fossa-Mastoid Neck approach
Large GlomusJugulare-Infratemporalfossaapproach
Tumoursextending towards Foramen Magnum -Transcondylarapproach

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