Tumours of

EarBRIG ANWAR UL HAQ00923018513303

Tumours of

Ear

Parts of Ear• External Ear• Middle Ear• Inner Ear

Parts of External Ear• Pinna• External auditory Canal– Cartilagenous– Bony

Tumours of Pinna• Carcinoma

– 85% Pinna– 5% External auditory Canal

Tumours of Pinna• Benign Tumours• Malignant Tumours

Benign Tumours of Pinna

Benign• Sebaceous Cyst• Preauricular Cyst or Sinus• Dermoid Cyst• Keloid• Haemangioma• Papilloma• Cutaneous Horn• Keratoacanthoma• Neurofibroma

Preauricular Sinus or Cyst• Results From Faulty Union of

– Hillocks of the 1 St and 2nd Branchial arches During the Development of Pinna

Dermoid Cyst• Usually Presents as a Rounded Mass Over the Upper Part of

Mastoid Behind the Pinna.

Keloid• Piercing of the Ear Lobule For ornaments Surgical Incision• Genetic Susceptibility• Black Races are More often affected• Keloid - Pedunculated Tumour

Haemangiomas• They are Congenital Tumours often Seen In Childhood• Capillary Haemangioma. • Mass of Capillary-sized Blood Vessels • Present as a "Port-wine Stain"• Does not Regress Spontaneously

Haemangiomas• Cavernous Haemangioma • (Strawberry Tumour)• Endothelial-lined Spaces Filled With Blood• Increases Rapidly During the First Year• Regresses thereafter • May Completely Disappear By the Fifth Year.

Papilloma (Wart)• Tufted Growth of Flat Grey Plaque • Rough to Feel• Viral In origin.• Treatment is Surgical Excision• Curettage With Cauterization

Cutaneous Horn• Heaping Up of Keratin• Presents as Horn -Shaped Tumour• it is often Seen at the Rim of Helix In

Elderly• Treatment is Surgical Excision

Keratoacanthoma• Benign Tumour• Resembles a Malignant One. • Raised Nodule with a Central Crater• Initially, it Grows Rapidly But • Slowly Regresses Leaving a Scar • Treatment is Excision Biopsy

Malignant Tumours• Squamous Cell Carcinoma• Basal Cell Carcinoma• Adenocarcinoma• Malignant Cercinoma• Melanoma

Malignant Tumours Pinna• Squamous Cell Carcinoma• Basal Cell Carcinoma• Adenocarcinoma• Malignant Carcinoma• Melanoma

Squamous Cell Carcinoma• Painless Nodule• Ulcer With Raised Everted Edges and Indurated Base. • Metastases to Regional Lymph Nodes Occur Very Late. • Common In Male +50years• Prolonged Exposure to Direct Sunlight.• Fair-complexioned People are More Prone.• Treatment. Small Lesions With No Nodal Metas Tases are• Excised Locally With 1 Cm of Healthy area around it.• Larger Lesions of the Pinna or Those Coming Within 1 Cm• of External auditory Canal and Lesions With Nodal Metastases, • May Require total amputation of the Pinna, often• With en Bloc Removal of Parotid Gland and Cervical Lymph• Nodes.

Treatment-SCC• Small Lesions With No Nodal Metastases are Excised Locally

with 1 Cm of Healthy Area Around it.• Larger Lesions of the Pinna or those Coming Within 1 Cm of

External auditory Canal and Lesions With Nodal Metastases, May Require total amputation of the Pinna often

• Bigger Tumours Removal of Parotid Gland and Cervical Lymph Nodes.

Basal Cell Carcinoma• Common Sites - Helix and the Tragus• >50 Years• Nodule With Central Crust,• Removal of Crest its - Bleeding. • Ulcer Has a Raised Beaded Edge• Extends circumferentially• May Confined to the Skin But • May Penetrate Deeper, Involving Cartilage or Bone • Lymph Node Metastases Usually Do Not Occur

Treatment - BCC• Radiotherapy• Superficial Lesions, Not Involving Cartilage• Cosmetic Deformity avoided• Surgical Excision• Lesions Involving Cartilage

External Ear Canal Benign• Osteoma• Exostosis• Ceruminoma• Sebaceous adenoma• PapillomaMalignant• Squamous Cell Carcinoma• Basal Cell Carcinoma• adenocarcinoma• Malignant Carcinoma• Melanoma

Osteoma• Arises From Cancellous Bone• Single, Smooth, Bony, Hard, Pedunculated Tumour• Often Arising From the Posterior Wall of the Osseous

Meatus• Treatment is Surgical Removal By– Fracturing Through its Pedicle – Removal With a Drill.

Exostosis• Smooth, Sessile, Bony Swellings In the Deeper Part of the Meatus• Multiple and Bilateral• Arise From Compact Bone • Exostosis - Exposed to Entry of Cold Water

– Divers – Swimmers

• Males are affected Three Times More

Exotosis - Treatment• No Treatment

– Small and asymptomatic• Larger Ones

– Impair Hearing– Retention of Debris

• Surgical Treatment• High Speed Drill to Restore Normal Sized Meatus• Use of Gouge and Hammer Should Be avoided

Sebaceous adenoma• Arises From Sebaceous Glands • Smooth, Skin Covered, Swelling In the

Outer Meatus. • Treatment • Surgical Excision.

Ceruminoma• Tumour of Modified Sweat Glands (Cerumenous)• Smooth, Firm, Skin-covered Polypoid Swelling • Usually attached to the Posterior or Inferiornwall • Obstructs the Meatus Leading to Retention of Wax and

Debris. • Malignant : Benign = 2: 1• Treatment. • Wide Surgical Excision• Tendency to Recur • Regular Followed Up

Malignant Tumours - SCC• Long-standing Ear Discharge • arise Primarily From the Meatus • May Be a Secondary Extension From the Middle Ear Carcinoma.• Presenting Symptoms• Discharge

– Blood Stained– Mucopurulent – Purulent Discharge

• Severe Earache• Examination

– Ulcerated area In the Meatus– Bleeding Polypoid Mass – Granulations – Facial Nerve Paralysis – May Spread Into the Middle Ear – Regional Lymph Nodes (Preauricular, Postauricular, Infra-auricular and Upper

Deep Cervical)

Treatment - SCC• En bloc wide surgical excision• Postoperative radiation.

Basal cell and adenocarcinomas

• Rarely arise from the meatus• Clinical picture is similar - SCCDiagnosis biopsy Treatment

Wide surgical excision Postoperative radiation

Ceruminoma• Malignant type is twice as common

as benign.

Malignant melanoma• Rare tumour

Middle EarMiddle

Ear

Primary Tumours• Benign: Glomus Tumour• Malignant: Carcinoma, SarcomaSecondary Tumours• from Adjacent Areas

– Nasopharynx, – External Auditory Meatus – Parotid.

Metastatic from Carcinoma of• Bronchus• Breast• Kidney • Thyroid• Prostate• Gastrointesinal Tract.

Tumours of Middle Ear

• From Adjacent Areas– Nasopharynx, – External AUDItoRY Meatus – Parotid.

Metastaticfrom Carcinoma of Bronchus

• Breast, • Kidney, • Thyroid, • Prostate• Gastrointestinal SYSTEM.

Secondary Tumours

• Most Common Benign Neoplasm of Middle Ear and is So-named Because of its Origin from the Glomus Bodies.

• Resemble Carotid Body in Structure • Location– Dome of Jugular Bulb – Promontory

• Along the Course of Tympanic Branch of IXth Cranial Nerve(Jacobson's Nerve)

• Paraganglionic Cells Derived from the Neural Crest

Glomus Tumour

• Age 40-50• Females : Male = 5:1• Characteristics

– Benign– Non-encapsulated– Extremely Vascular

• Rate of Growth is Very Slow • Tumour is Locally Invasive.• Microscopically

– Masses Or Sheets of Epithelial Cells• Large Nuclei • Granular Cytoplasm.• Thin-walled Blood Sinusoids With• No Contractile Muscle Coat - Profuse Bleeding

Aetiology and Pathology

Glomus JugulareArise from the Dome ofjugular Bulbinvade the Hypotympanum and Jugular Foramen,Ixth to Xlith Cranial Nerve involvementCompress Jugular Veininvade its Lumen .

Glomus TympanicumArise from the Promonory of the Middle Ear Cause Aural SymptomsAural SymptomsFacial Paralysis.

Types of Glomus Tumours

• Initially Fill the Middle Ear• Later Perforate Through the Tympanic Membrane - Vascular Polyp.• May invade

– Labyrinth– Petrous Pyramid – Mastoid– Jugular Foramen– Base of Skull

• Causing Ixth to Xllth Cranial Nerve Palsies.• My Spread Through

– Eustachian Tube, It May Present in the Nasopharynx.– Intracranially to the Posterior and Middle Cranial Fossae.

• Metastatic Spread– Lungs and Bones is Rare - 4% – Metastatic Lymph Node Enlargement

Spread of Glomus Tumour

• I9% of Cases, - Symptoms Pertain to the Ear.• Umour is intra Tympanic - Earliest Symptoms

– Conductive Deafness – Pulsatile Tinnitus - Stops With Carotid Pressure

• Otoscopy – Red Reflex Through intact Tympanic Membrane - "Rising Sun– Bluish and May Be Buging.– "Pulsation Sign" (Brown's Sign) Ear Canal Pressure is Raised With Siegle's

Speculum, • Tumour Pulsates Vigorously and then Blanches; Reverse Happens With Release of

Pressure.

• Polyp - Profuse Bleeding• Dizziness Or Vertigo • Facial Paralysis• Earache - Less Common • Otorrhoea - infection and

– May Simulate Chronic Suppurative Otitis Media With Polyp.

Clinical Features

• IXth to XIIth Cranial• Dysphagia • Hoarseness• Unilateral Paralysis of

– Soft Palate– Pharynx– Vocal Cord – Weakness of the Trapezius and Sternomastoid Muscles.

• Mass Over the Mastoid or in the Nasopharynx.• Signs of intracranial involvement• Audible Systolic Bruit• Secrete Catecholamines and Produce Symptoms Like

– Headache, Sweating, Palpitation, Hypertension and Anxiety• 10% of the Tumours - Familial• 10% - Multicentric • 10% - Secrete Catecholamines

Cranial Nerve Palsies

• CT Scan• MRI• Angiography

Diagnosis

• Surgical Removal.• Radiation.• Embolisation.• Combination of the Above Techniques

Treatment

• Rare Condition • Age Group of 40-60• Slightly More Common in Females• 75% - Ear Discharge• Chronic Irritation - Causative Factor• Radical Mastoid Cavities• Primary Carcinoma of Mastoid Air Cells • Radium Dial Painters.

Carcinoma of Middle Ear and Mastoid

• Destroys – Ossicles– Facial Canal– internal Ear– Jugular Bulb, Carotid Canal – Deep Bony Meatus– Mastoid– Pctrous Apex. – Dura is Usually Resistant

• Other Structures– Parotid Gland– Temporomandibular Joint– infratemporal Fossa – Eustachian Tube– Nasopharynx

• Lymph Node Enlargement Occurs Late.

Spread of Tumour

• Like CSOM Discharge and Deafness• Females 40- 60 Suspicion– Chronic Foul-smelling Discharge– Blood-stained.

• Pain Severe and Comes At Night.• Facial Palsy• Friable, Haemorrhagic Granulations Or Polyp.• Deafness• Vertigo

Clinical Features

• Definitive Diagnosis is Made only on Biopsy.• Extent of Disease– Cranial Nerve Palsies– Radiological Examination.

• CT Scan and Angiography are Useful in the Assessment of Disease.

Diagnosis

• Surgery• Radiotherapy

Treatment

• Rhabdumyosarcoma • Osteosarcoma• Lymphoma• Fibrosarcoma• Chondrosarcoma• Distant Metastases– Lungs – Bone

Other Tumours

• Synonyms– Vestibular Schwannoma– Neurilemmoma – Eighth Nerve Tumour

Acoustic Neuroma

• 8O% - Cerebellopontine Angle Tumours• 10% - All the Brain Tumours.

incidence

• Benign• Encapsulated• Extremely Slow-growing• Microscopically– Elongated Spindle Cells – Rod-shaped Nuclei – Cell Lie Rows Or Palisades– Bilateral Tumours - With Neurofibromatosis.

Pathology

• Schawan Cells

Origin

• Depending on the Size• (A) intracanalicular (Confined to IAC)• (B) Small Size (Up to 1.5 Cm)• (C) Medium Size (1.5 to 4 Cm)• (D) Large Size (Over 4 Cm)

Classification

• 40-60 Years• Male:female = 1:1

Clinical Features

• Earliest Symptoms - Still intracanalicular – Pressure on Cochlear Or Vestibular Nerve

• Progressive Unilateral Sensorineural Hearing Loss, • Tinnitus• Speech Discrimination Is Poor– Out of Proportion to the Pure tone Hearing Loss.

• May Get Sudden Hearing Loss.• Imbalance Or Unsteadiness. • True Vertigo is Seldom Seen

Cochleovestibular Symptoms

• Vth Nerve Earliest Nerve - 2.5cm -CP Angle– Reduced Corneal Sensitivity– Numbness – Paraesthesia of Face

• VIIth Nerve. – Sensory Fibers Affected Early - Hypoaesthesia of Posterior Meatal Wall – Loss of Taste – Reduced Lacrimation on Schirmer's Test. – Motor Fibers Are More Resistant and Are Affected Late.

• IXth and Xth Nerves – Dysphagia – Hoarseness

• Other Cranial Nerves. • XIth and XIIth, Vllrd, IVth and VIth - Very Large.

Cranial Nerve involvement

• Ataxia• Weakness• Numbness of the Arms Legs• Exaggerated Tendon Reflexes

Brainstem involvement

• Pressure Symptoms on Cerebellum Are Seen in Large Tumours. This is Revealed By

• Finger-nose Test• Knee-heel Test• Dysdiadochokinesia• Ataxic• inability to Walk Along A Straight Line

Cerebellar involvement

• Late Features• Headache• Nausea• Vomiting• Diplopia - Vlth Nerve involvement • Papilloedema - Blurring of Vision

Raised intracranial Tension

• Pure tone Audiometry • Speech Audiometry - Poor Speech Discrimination• Reduction of Discrimination Score When Loudness is increased• Recruitment Phenomenon is Absent.• Short increment Sensitivity index (Sis!) Test Will Score of 0-

20% in 70-90% of Cases.• Threshold tone Decay - Retrocochlear Lesion• Stapedial Reflex Decay• Evoked Response Audiometry• MRI With Contrast

Audiological Tests

• Meniere's Disease) • Tumours of Cerebellopontine Angle• Acoustic • Meningioma• Epidermoid (Cholesteatoma)• Arachnoid Cyst• Schwannoma of Other Cranial Nerves• V, VII, IX, X, XI• Aneurysm• Glomus Tumour• Metastatic Tumours

Differential Diagnosis

• Surgery• Surgical Removal of the Tumour is the Treatment of

Choice.• Surgical Approach Will Depend Upon the Size of

Tumour.• The Various Approaches Are:• 1. Middle Cranial Fossa Approach.• 2. Translabyrinthine Approach .• 3. Suboccipital Approach.• 4. Combined Translabrynthine-Suboccipital Approach

Treatment

• Radiotherapy• Conventional Radiotherapy By External Beam

Has No Role in the Treatment of Acoustic Neuromas due to Low tolerance of the Central Nervous System to Radiation.

Treatment

• Gamma Knife Surgery• Stereo-tactic Radiotherapy • Radiation Energy is Converged on the Tumour• Minimum Effects on the Surrounding Tissue.• This Causes

– Arrest of the Growth of the Tumour – Reduction in its Size

• Indications– Refuse Surgery – Contra indications to Surgery – Residual Tumour

Treatment

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