Neoplasms of the ear

Department of ENT
SIMS &RC
Classification
Malignant
Benign
 Glomus tumors
Squamous cell ca.
 Acousticmalignancy
Parotid neuroma
 Salivary tumors
 Osteoma/ exostosis
 Ceruminoma
Definition

 A benign slow growing tumor

 Arising from the schwann cells of the
sup/inf. Vestibular nerve
 In the internal acoustic meatus later
involving CP angle
 Symptoms, signs and complications by
virtue of its topographical situation
Incidence

 8-10% of all brain tumors

 80% of cerebello-pontine angle tumors
Tumor behavior
 Usually arises from the superior vestibular
nerve in the IAM
 Rarely from the inferior vestibular nerve/
cochlear nerve
 Arises from the schwann cells and hence
tumor seen at the periphery of the nerve.
Internal acoustic Tumor
meatus
Nerve
Type A
(Intracanalicular tumor)
 Tumor confined to the
IAM
 As it increases in size
it expands and
erodes the bone
 Compresses other
nerves in the IAM
Type B
 Grows into CP angle
 Less than 2.5 cms. in
diameter
 Medium size

Blunting if the
posterior lip
Type C Brain stem
Trigeminal N.
 Large tumors
 More than 2.5 cms.
 Displaces the brain
stem and cerebellum
 Contact/ compress
the trigeminal and/ or
the abducent nerves
 Inferiorly may involve
IX, X, XI nerves
Cerebellum
Pathology- Gross
 Benign, well encapsulated, firm, whitish
 Nerve is at the periphery of the tumor, not
in its center
 May be associated with arachnoid cysts
due to secondary arachnoiditis
 Rarely cystic due to central degeneration
or hemorrhage into the tumor
Pathology- Microscopy
 2 types
A. Fasciculated type (Antony A type)
 Cells arranged orderly in parallel rows
 Interwoven bundles with nuclei and fibrous
zones (Pallisading pattern)
B. Reticular type (Antony B type)
 Disorderly arranged loose network of cells
 Intercellular vacuoles and cysts
 Haemorrhage within tumor can give rise to
sudden increase in size
CLINICAL FEATURES
SYMPTOMS
 Age: 30-50 years (4th decade)
 Sex: Equal M:F::3:2
 Unilateral in 90% of cases
 Bilateral in 10%-Von Recklinghausen’s
disease
 Presentation depends on the stage
 Vestibulocochlear CN Brain stem
ICT
Otological symptoms
 Caused by pressure on VIII N in IAM
 Cochlear:
 Progressive, unilateral deafness with tinnitus
 Poor speech discrimination
 Rarely- sudden deafness
 Vestibular:
 Mild due to slow growth and compensation
 Imbalance/ occasional vertigo/ secondary Meniere’s
disease
Cranial Nerve palsy
 V
 First CN after VIII
 Pain, numbness and paresthesia of the face
 Indicates tumor more than 2.5 cms. (Stage C)
 VII
 VII N palsy late as it a motor nerve- more resistant
 Sensory fibres first- Motor next
 Hypoesthesia of the postero-superior meatal wall
(Hitselburger’s sign)
 Loss of taste/ reduced lacrimation/ delayed blink
Cranial N. palsy- contd.
 IX, X, XI
 In Type C
 Dysphagia
 Hoarseness
 Nasal regurgitation
 VI, III
 Rare
 Diplopia
Brain stem/ cerebellar
involvement

 Ataxia
 Weakness of arms/ legs
 Imbalance- tendency to fall on the
affected side
 Tremors
Increased ICT
 Headache
 Nausea and vomiting
 Blurring of vision
SIGNS
 TM normal- TF tests: SN loss
 Hitselburger’s sign
 Nystagmus +/-, Ataxia +/-
 Fistula sign –ve
 CORNEAL REFLEX: May be absent (50%)
 Palatal/ vocal cord palsy
 Exagerrated tendon reflexes
 Finger-nose test
 Diplopia/ <taste/ < lacrimation
 Papillodema
Investigations- Objectives
 Diagnose at the early stage- ‘A’
 Clinical suspicion- investigate to
 Confirm diagnosis
 Assess the extent
 Relation to brain stem/ cerebellum
 Early treatment
 Morbidity
 Mortality
Audiological tests
 PTA- SN loss- unilateral
 Speech audiometry: <SD scores (0-30%)
 Recruitment: absent
 Tone decay: Positive
 Stapedial reflex decay: Positive in 65%
 BERA: Latency I-V delayed (95%)
Vestibular function tests
 Nystagmus: +/-
 Caloric test: Canal paresis positive
 Positional/ Rotational tests
 Electronystagmography: document the
spontaneous/ induced nystagmus
Radiological tests

 Plain X-rays: Transorbital/ Stenver’s / Towne’s

views
 Multidirectional tomography
 CT scan- high resolution- with magnification and
contrast- Type A may be missed
 MRI with gadolinium- Most accurate
 Vertebral angiography
 Cisternography+ CT scan
Treatment
 SURGICAL EXCISION
 Wait and watch policy in elderly patients
> 60 years- if tumor is small and no
symptoms
SURGICAL APPROACHES
 Type A and B: Microsurgical approaches
 Middle cranial fossa approach
 TRANSLABYRINTHINE APPROACH
 Type C: Neurosurgical approach
 Suboccipetal approach
 Other approaches:
 Transotic
 Presigmoid- retrolabyrinthine
 Retrosigmoid
Neurosurgical approach
 Need to retract brain
 Increase morbidity
 AICA bleeding
Translabyrinthine approach
 Microsurgical
 Less morbidity
 No dural/ brain retraction
 Hearing loss +, hence done only when SN
loss is present
 CUSA
Middle cranial fossa approach
 Ideal for type A
 Microsurgical
 Less morbidity
 Hearing, VII N preserved
 Difficult for large tumors
 Stereotactic Radiation Therapy
(Radio surgery):
• A single relatively high dose of radiation
delivered precisely to a small area will
arrest or kill the tumor
• Minimal injury to the surrounding nerves
& brain tissue
PROGNOSIS
 Small lesions
 Good prognosis/ less morbidity
 Large lesions
 Poor prognosis/ more morbidity
 Involving brain stem/ cerebellum/
Increased ICT
 Very poor prognosis
Glomus tumors

 Syn:
 Chemodectoma
 Non-chromaffin paraganglioma
 Definition:
 Defined as histologically benign but locally
invasive, highly vascular tumors arising
from the glomus bodies (chemoreceptors)
in relation to the ear.
Types
 Glomus jugulare
 Glomus bodies along the jugular bulb
 Floor of the middle ear
 Glomus tympanicum
 Glomus bodies along the tympanic plexus
formed by the IX CN (Jacobson’s nerve)
 Medial wall of the middle ear
 Pathology
 Tumor erodes the bone
 Upwards- middle ear-
perforation-aural polyp
 Downwards- jugular
foramen- involve IX, X, XI
cranial nerve, rarely XII
 Medially- labyrinth, facial
N, petrous apex-V and VI
cranial nerves
 Intracranial
 Extension to neck along
jugular vein
Paraganglioma (chaemodectoma) (HE x100). Typical cellular
groups ("Zellballen") surrounded by a capillary network and
nerve fibrils. Round, fairly regular nuclei. Variable, often granular
eosinophilic cytoplasm.
Symptoms
 >Middle aged-elderly females
 Pulsatile tinnitus- may reduce or disappear
on jugular occlusion
 Blood stained otorrhoea
 Unilateral hearing loss
 Ear ache- rare
 Neurological
Signs
 ‘Sun rise’ sign
 ‘Browne’s sign’-
blanching on
siegalization
 Aural mass- bleeds
on touch
 Bruit
 Neurological

Glomus jugulare
Glomus tympanicum
Investigations
 Audiological
 Radiological
 Plain radiographs of the mastoids
 CT scan with contrast
 Jugular venography
 Carotid and vertebral angiography- ascending
pharyngeal artery usually- embolization can be done
 Biopsy- done with caution
Pre and post-embolization
Treatment
 Treatment of choice- SURGICAL
 Approach depends on the extent of the tumor
 Tympanotomy
 Extended facial recess approach
 Canal down Mastoidectomy
 Subtotal temporal bone resection with or without
craniotomy
 Radiotherapy for unresectable tumors,
residual tumors or those with increased
morbidity suspected due to surgery
(Neurological)
 Supportive treatment for neurological
complications