VSS MEDICAL COLLEGE, BURLA

GLOMUS TUMOURDR UTKAL MISHRA

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.

SYNONYM

Chemodectoma

Paraganglioma

Ganglia Tympanica

Vascular tumour of middle ear

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.

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.

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

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.

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

TYPES

2 types according to site of origin –

1. GLOMUS TYMPANICUM – Arising from promontory.

2. GLOMUS JUGULARE – Arising from dome of jugular bulb.

RULE OF 10

10 % Multicentric

10 % Familial

10 % Functional

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.

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.

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.

CLASSIFICATION

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

LUNDGREN CLASSIFICATION

Glomus Tympanicum

Glomus Jugulare

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

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.

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 canalType 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

DIFFERENTIAL DIAGNOSIS

Otitis Media Otosclerosis Cholesterol Granuloma Aberrant Intrapetrous Internal Carotid

Artery Idiopathic Hemotympanum Aneurysm Arteriovenous Malformation Prominent jugular bulb Persistent stapedial artery

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)

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.

TREATMENT

Treatment of choice – Microsurgical total tumor removal after pre – op embolization of feeding vessel.

Inoperable case - Radiation

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

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

MODIFIED DE LA CRUZ CLASSIFICATION

TRANSCANAL APPROACH

EXTENDED FACIAL RECESS APPROACH

MASTOID NECK APPROACH

MASTOID NECK APPROACH

MASTOID NECK APPROACH

MASTOID NECK APPROACH

PRESENT SCENARIO

There is no MRI No Interventional Radiologist No Operating Microscope No Light Source !!!!

Only management → URGENT REFERRAL

THANK YOU