Tumours of External Auditory Canal and Middle Ear

TUMOURS OF EXTERNAL
EAR AND MIDDLE EAR
Dr Harjitpal Singh
Assistant Professor(ENT),
Dr RKGMC, Hamirpur

EXTERNAL EAR
• External Ear comprises of the
Auricle and
External Auditory canal.
• Of all the cases of Ear carcinoma,
85% occur on the pinna,
10% in the external canal and
5% in the middle ear.

TUMOURS OF AURICLE- BENIGN
Pre-auricular Sinus or Cyst:
• Due to faulty union of
hillocks of 1st and 2nd
branchial arches
• Tract lined by squamous
epithelium

• Sebaceous cyst – post
auricular sulcus
• Dermoid cyst – over upper
part of mastoid
• Keloid – follow trauma.
Treatment comprises of:
• Surgical excision and
• Injection of triamcinolone.

Hemangioma:
• 1.Capillary – port wine
stain. It does not regress
spontaneously.
• 2.Cavernous – also called
strawberry tumor. It
increases rapidly during
the first year but
regresses thereafter and
may completely
disappear by the fifth
year.

TUMOURS OF AURICLE(cont)
PAPILLOMA(WART):
It may present as a
tufted growth or flat grey
plaque and is rough to
feel. It is viral in origin.
Treatment is surgical
excision or curettage with
cauterization of its base.

TUMOURS OF AURICLE-MALIGNANT
SQUAMOUS CELL CA:
• Helix
• Painless nodule or ulcer
• Common in Males in fifties .
• Small lesions – excised locally
• Large lesions with nodal
mets – total amputation of
pinna with en bloc removal
of parotid and cervical nodes

BASAL CELL CA(Rodent Ulcer):
• Men beyond 50 Years.
• This type usually presents
initially as a raised
pigmented plaque with a
tendency to form crusts.
• Common sites involved are
the border of helix, meatal
entrance and the tragus
• No lymphatic spread
• Superficial lesions –RT
• Deep - Surgery

MALIGNANTMELANOMA:
• Light complexion
• Exposure to sunlight
• Patient presents with a brownish
black nodular lesion on pinna,
which grows rapidly and may
ulcerate
• Lymph nodes are usually involved
and distant metastasis may take
place.
• Superficial <1cm –wedge
resection
• Radical excision with block
dissection of lymph nodes.
Prognosis is not good.

TUMOURS OF EAC- BENIGN
EXOSTOSES:
• Exostoses present as hemispherical smooth bony
outgrowths from the canal wall.
• These are usually multiple and the condition is usually
bilateral. The exact cause is not known but it was thought
that repeated swimming in cold water could be an
aetiological factor.
• Exostoses usually do not produce any symptoms unless
these outgrowths obliterate the lumen of the canal.
• When exostoses are small and symptomless, no treatment
is needed.
• But when these outgrowths become large in size, they are
removed using a drill and cutting burr.

OSTEOMA;
• Benign tumours consisting of mature lamellar
bone
• Causes conductive hearing loss.
• It presents as a single,smooth,bony hard
pedunculated tumour often arising from the
posterior wall of osseous meatus near its outer
end.
• RX is surgical removal by fracturing through its
pedicle or removal with a drill.

CERUMINOMA:
• It’s a tumour of modified sweat glands which secrete
cerumen.
• Presents as smooth, firm, skin covered polypoid swelling
in outer parts of meatus generally attached to the
posterior or inferior wall.
• Obstruct the meatus retaining wax and debris.
• Malignant outnumber benign by 2:1 ratio.
• RX tumour has a tendency to recur, so wide surgical
excision should be done and patient regularly followed
up.
• Malignant ones should be given radiotherapy.

SEBACEOUS ADENOMA:
• Arise from sebaceous glands of meatus
• Present as smooth,skin covered swelling in the outer
meatus
• RX is surgical excision.
PAPILLOMA:
• Presents as a tufted growth or flat grey plaque and is
rough to feel.
• Viral in origin
• RX is surgical excision or curettage withcauterisation of
its base.

TUMOURS OF E.A.C- MALIGNANT
SQUAMOUS CELL CARCINOMA:
• Most common malignant tumour
• Most often seen in cases of chronic ear discharge.
• May arise primarily from the meatus or be a
secondary extension from the middle ear
carcinoma
• Presenting symptoms are blood staining of
mucopurulent or purulent ear discharge and
severe earache.
• Examination shows an ulcerated area in the
meatus or a bleeding polypoid mass or
granulations

SQUAMOUS CELL CARCINOMA(C0NT)
• Facial nerve may be paralysed due to local extension
through post meatal wall or its spread into the middle ear.
Males effected three times more than females.
• When small, no RX is needed. Larger ones which impair
hearing or cause retention of wax or debris,may be
removed with high speed drill to restore normal sized
meatus.
• May extend deeply and lie in deep asssociation with
facial, so use of gouge and hammer should be avoided.
• Regional lymph nodes ie preauricular, postauricular,
infraauricular and upper deep cervical may be involved.
• RX is enbloc wide surgical excision and postoperative
radiation.

BASAL CELL AND ADENOCARCINOMA:
• Rarely arise from the meatus
• Clinical picture similar to squamous type
• Diagnosis is made on biopsy only
• RX is wide surgical excision and postop
radiation.

MALIGNANT CERUMINOMA
• Twice as common as benign
MALIGNANT MELANOMA
• A very rare tumour.

GLANDULAR TUMOURS OF E.A.C-BENIGN
CERUMINOUS ADENOMA:
• These are well differentiated tumours derived from
ceruminous glands which exhibit solid,cystic and
papillary patterns.they consist of an inner layer of cells
that are cuboidalor columnar and of apocrine origin
with an outer myoepithelial in origin.these are widely
excised as recurrence is common.no role of
radiotherapy here.
PLEOMORPHIC ADENOMA:
• It is also excised. Seedling should be avoided by
spillage during resection.recurrence common here too.

GLANDULAR TUMOURS OF E.A.C- MALIGNANT
ADENOID CYSTIC CARCINOMA:
• Displays cribriform,tubular or solid growth
patterns sorrounded by cystic spaces.
• Has the propensity for local tissue destruction,
perineural and perivascular invasion.
• Can metastasize to lymph nodes of neck
• Best managed by aggressive surgical resection
followed by radiotherapy.
• Overall poor prognosis.

GLANDULAR TUMOURS OF E.A.C- MALIGNANT
CERUMINOUS ADENOCARCINOMA:
• Difficult to distinguish from ceruminous adenoma
as it may show very few malgnant features apart
from invasion into local tissue.
• Slow growing and has local invasion.
• Metastasis is reported.
• Rare tumour
• Surgical resection with postop radiotherapy
choice of treatment.

TUMORS OF THE MIDDLE EAR AND MASTOID
Tumours of middle ear and mastoid can be divided into:
1. Primary tumours
(a) Benign: Glomus tumour
(b) Malignant: Carcinoma, sarcoma
2. Secondary tumours
(a) From adjacent areas, e.g. nasopharynx,
external meatus and the parotid.
(b) Metastatic, e.g. from carcinoma of bronchus,
breast, kidney, thyroid, prostate and
gastrointestinal tract.

• Malignancies of middle ear and mastoid are very rare conditions.
• Incidence may be 0.006 per thousand cases seen in ear, nose and
throat (ENT) outdoor and the usual age group is40 to 60 years
• Females are affected more commonly and there is a history of
long-standing ear discharge of 20 years duration causing constant
irritation
• Persons working in radium dial painting in watch companies, may be more
prone to carcinoma of the middle ear
• Histologically, squamous cell carcinoma is the mostcommon type of
carcinoma followed by adenocarcinoma and sarcoma
(rhabdomyosarcoma)
• Carcinoma of the middle ear may spread through natural cavities to the
facial canal; internal ear; deep bony meatus, petrous apex;
• Tumors of the ear may masquerade as chronic ear infection. While
treating these chronic EAC and middle ear infections, the suspicion
of neoplasm should be kept in mind.

- BENIGN
GLOMUS TUMORS (PARAGANGLIOMAS):
• This most common benign neoplasm of middle ear arises
from the glomus bodies, which resemble carotid body
in structure.
• The paraganglionic cells of the tumor are derived from the
neural crest.
• It is also called chemodectoma, because it resembles
carotid bodies
• These cells produce catecholamines and neuropeptides
serving as neurotransmitters.
• Though the tumor is locally invasive, its growth is very slow,
extending over several years.
• They carry < 3% malignancy conversion rate.

- BENIGN
Etiopathogenesis
• Commonly seen between 40 and 50 years of age
• The male:female ratio is 1:5
• It is slow growing, benign, noncapsulated, locally
invasive and extremely vascular tumor.
Histopathology
• Shows thin-walled vascular sinusoids with no
contractile muscular coat and plenty of epitheloid
cells with large oval nuclei and granular
cytoplasm.

- BENIGN
Sites
• The glomus bodies are present in the dome of jugular bulb
and along the course of tympanic branch of 9th cranial
nerve (Jacobson’s nerve) on the promontory.
• So there are mainly two types of glomus tumors:
1. Glomus Jugulare: This tumor arises from the dome of
jugular bulb and invades the hypotympanum and
jugular foramen. It involves cranial nerve, CN Ix,
x, xI and xII and may compress jugular vein and
invade its lumen.
2. Glomus Tympanicum: This tumor arises from the
promontory and may cause facial paralysis.

- BENIGN
Spread
• „Local: The tumor first fills the middle ear and then invades the
tympanic membrane and present as an ear polyp, which
bleeds readily.
• It may later on invade following structures:
labyrinth, petrous pyramid & the mastoid.
Jugular foramen & the 9th to 12th cranial nerves.
Through Eustachian tube, tumor may enter into the
nasopharynx.
Posterior and middle cranial fossa.
• Metastatic spread to lungs and bones is rare, but seen in 4% of
cases. Metastatic lymph node enlargement can also occur.

- BENIGN
Clinical Features:
a. Intratympanic Tumor(Otological symptoms)
• Earache, diminished, progressive conductive hearing loss.
• Pulsatile tinnitus, which stops on pressing the carotid artery,
imbalance and blood-stained discharge.
Otoscopy shows Rising sun sign behind the bluish tympanic
membrane. ‘Brown’s sign’ is seen when pressure is
applied with Siegle’s speculum, tumor pulsates and
even blanches on increasing the pressure.

- BENIGN
Clinical Features:
b. Ear Polyp:
• Profuse bleeding: Spontaneously or on attempts to clean EAC.
• Dizziness or vertigo.
• Facial paralysis.
• Earache is less common than in carcinoma of the external and
middle ear.
• Otorrhea: It is due to secondary infection and simulates chronic
suppurative otitis media polyp.
• Red, vascular polyp filling the meatus, which bleeds readily and
profusely on manipulation.
• Biopsy is contraindicated.

- BENIGN
Clinical Features:
c. 9th to 12th Cranial Nerve Palsies
This is a late feature appearing several years
after aural symptoms.
• Dysphagia.
• Dysarthria and hoarseness of voice.
• Unilateral paralysis of soft palate, pharynx and
vocal cord.
• Weakness of the trapezius and sternomastoid
muscles (XI) and atrophy of half of tongue
(XII).

- BENIGN
Clinical Features:
d. Catecholamine features: Headache, sweating,
palpitation, hypertension and anxiety.
e. Mass over the mastoid or in the nasopharynx.
Rule of 10s. Remember that 10% of the tumours are
familial, 10% multicentric (occurring in more
than one site) and up to 10% functional, i.e.
they secrete catecholamines.

- BENIGN
Differential Diagnoses
Because of their appearance, glomus tumors may be
mistaken for:
• High-riding jugular bulb or dehiscent jugular
bulb.
• Aberrant carotid artery.

- BENIGN
Investigations
Catecholamines:
Serum levels of catecholamines.
Break-down products of catecholamines in urine:
Vanillylmandelic acid, metanephrine,
CT scan-head: Bone window, 1 mm thin sections help to distinguish
glomus tympanicum from the glomus jugulare. Caroticojugular
spine is eroded in the glomus jugulare. The aberrant carotid
artery, high or dehiscent jugular bulb can also be diagnosed.
„Magnetic resonance imaging: Magnetic resonance imaging (MRI)
provides soft tissue extent of the tumor.
„Magnetic resonance angiography and venography: Magnetic resonance
angiography (MRA) and venography delineate invasion of jugular
bulb and vein or compression of then carotid artery.
„.

- BENIGN
Investigations
Four vessel angiography: It provides following
information:
Extent of tumor
Compression of internal carotid artery
Finding other carotid body tumors
Decision for embolization of tumor
Brain perfusion studies and adequacy of
contralateral internal carotid
artery and circle of Willis.
„Biopsy: It is contraindicated because the tumor is highly
vascular and bleeds profusely.

AXIAL CT SCAN SHOWING GLOMUS TUMOUR
GROWING FROM MEDIAL WALL

CORONAL CT SCAN SHOWING GLOMUS
TUMOUR GROWING FROM THE MEDIAL WALL
OF MIDDLE EAR.

- BENIGN
Management
The treatment depends on diagnostic evaluation
along with age, type of tumor and general physical health.
Palliative treatment
Palliative treatment is given to elderly patients of 65 to 70 years, medically
infirm or those patients having multicentric lesions.
Radiation therapy
Radiation therapy is given because of low morbidity, low cost and
conservative therapy or to patients with unresectable tumors or
to control the vascularity of tumors. Side effects, such as hearing
loss, osteoradionecrosis, central nervous system (CNS) damage
and radiation-induced malignancy, must be kept in mind.
Embolization
Embolization reduces the vascularity of tumor before surgery. It may
become the sole treatment in inoperable and irradiated patients.

- BENIGN
Management
Surgical treatment
• The approach may be transmeatal tympanotomy (for Type I)
• Transmastoid with extended facial recess approach (for Type II)
or
• Skull base approach for advanced lesions. Infratemporal
fossa approach of Fisch is good for large tumors.
In skull base approaches, identification and
monitoring of facial nerve and internal carotid artery is vital.
Intracranial extensions may have to be managed with the
assistance of a neurosurgeon.

- BENIGN
OTHER BENIGN TUMORS
Benign tumors are not very commonly seen. Some of
the tumors seen are adenoma, hemangioma, osteoma,
neurinoma of facial nerve, acoustic nerve or IXth and Xth
nerve.
These are very slow growing tumors causing blockade, deafness
and nerve palsies.
Diagnosis is confirmed by tympanotomy and histopathology
with wide excision of the mass.

- BENIGN
Inverted papilloma
• Rare tumours of middle ear
• HPV is implicated in pathogenesis.
• Surgery is the mainstay of treatment.
Haemangioma
• Benign vascular tumours derived from
arterioles,venules,and capillaries.
• Geniculate ganglion is the most common site.
Present with conductive hearing loss.
Some may regress spontaneously while others continue to
grow and become locally destructive.
Surgical excision is the T.O.C

- Conditions Simulating Benign Tumors
Fibrous Dysplasia
• Fibrous dysplasia is a benign tumor like lesion
of bone, rarely affecting the external auditory
canal wall and middle ear.
• It may be of monostotic or polyostotic type.
• Once the diagnosis is confirmed by biopsy, the
treatment is by wide incision.

Lipoid Dystrophies
1. Letterer-Siwe disease: It occurs under 2 years of age and is
a fatal disease.
2. Hand-Schüller-Christian disease: It occurs in 10 to 20 years
of age group and the patient presents with diabetes
insipidus; exophthalmos and defect in skull bones. X-ray
shows erosion of bone. Histology shows lipid-filled
histiocytes. Treatment is radiotherapy.
3. Eosinophilic granuloma: This condition is seen in children,
the frontal bone is commonly involved and the
symptoms resemble to acute osteomyelitis. Histology
shows mononuclear histiocytes with mitotic figures.
Treatment is excision followed by radiotherapy.

Wegener’s Granuloma
Wegener’s granuloma is a systemic disease involving
mainly the nose, nasopharynx, lungs and kidney,
the etiology of which is not known.
It is very rarely seen in ear, but when present, patient
presents with blood-stained ear discharge,
granulations, lot of destruction of external auditory
canal and the middle ear structure.
Biopsy from the tissue shows epithelioid granuloma and
necrotizing vasculitis.
Treatment is by systemic steroids and cytotoxic drugs.

- MALIGNANT
Carcinoma of middle ear/EAC
Carcinoma of middle ear and mastoid, though the
commonest primary middle ear malignancy is rare tumor. It
arises either primarily from middle ear or is an extension of
carcinoma of deep bony meatus.
Etiology
1. Age: Patients are in the age group of 40–60.
2. Sex: It is slightly more common in females.
3. Chronic suppurative otitis media: Most patients (75%) have
associated chronic ear discharge that mimics chronic
suppurative otitis media (CSOM). Chronic irritation is perhaps
the causative factor. It can develop in radical mastoid cavities
4. Radium dial painters: Primary carcinoma of mastoid air cells is seen.

- MALIGNANT
Pathology
• „Types of carcinoma of EAC in order of decreasing
frequency include squamous cell carcinoma,
basal cell carcinoma and adenoid cystic
carcinoma.
• „Adenocarcinoma, which is less common, arises from
the glandular elements of middle ear.

- MALIGNANT
Spread
• The tumor destroys ossicles, facial canal, internal ear, jugular
bulb, carotid canal, deep bony meatus and mastoid.
• „Medially it spreads toward the petrous apex. Dura is usually
resistant to the spread.
• „Anteriorly it spreads and involves parotid gland,
temporomandibular joint and infratemporal fossa.
• „Through the Eustachian tube it can enter into the
nasopharynx.
• „Lymph nodes are involved at an advanced stage.

- MALIGNANT
Clinical features
• Appearance of persistent and inordinate pain or blood stained discharge in
chronic cases of painless mucopurulent or purulent otorrhea (CSOM or
chronic otits externa) should raise the suspicion.
• „Severe ear pain especially in night.
• „An ulcerated area in the meatus or a bleeding friable polypoid mass or
granulations.
• „Meaty or polypoid mass in EAC (squamous cell carcinoma)
• Small pimple with significant pain (adenoid cystic carcinoma).
• „Serpiginous ulceration (basal cell carcinoma).
• „Facial nerve palsy can occur because of local extension
• „Labyrinthine involvement causes SNHl and vertigo.
• „Late features: Parotid mass, CN Ix-xII palsy, cervical lymphadenopathy.
• Tumor may originate from nasopharynx.
• „Enlargement of regional lymph nodes: Preauricular, postauricular, infra-auricular
and upper deep cervical.

Carcinoma left middle ear and external auditory canal
(EAC). Mass in left EAC. Inset shows left facial palsy of the same
patient

Squamous cell carcinoma of the
middle ear and mastoid.

- MALIGNANT
Investigations
„CT scan and angiography: They show the extent of
tumor and bone invasion
„Biopsy: Biopsy should be taken in cases of
intractable case of chronic OE.
Histopathology will confirm the nature of lesion.

- MALIGNANT
Treatment
It consists of en bloc wide surgical excision with postoperative
radiation.
The combination of surgery and radiotherapy offers better
prognosis.
„Surgery: Depending on the extent of tumor, it may consist
of radical mastoidectomy, subtotal/total petrosectomy.
„Radiotherapy: Radiotherapy alone is a palliative measure
• and is considered when the growth involves CN Ix, x, xI
and xII or spreads to the cranial cavity or nasopharynx.
It is given in doses of 5,000 to 7,000 rads

- MALIGNANT
Rhabdomyosarcoma
This rare tumor arises from the embryonic muscles,
tissue or the pluripotential mesenchyme. It mostly affects children.
Clinical features
a. Early cases mimic CSOM and have ear discharge, polyp or
granulations.
b. Facial palsy occurs early.
c. Swelling in the surrounding region of the ear.
„Biopsy: It establishes the diagnosis.
„Treatment: A combination of radiation and chemotherapy is the
best modality of treatment. Surgery is considered in selected
localized tumors.
„Prognosis: It is poor.

Rhabdomyosarcoma of The Right
Middle Ear And Mastoid

- MALIGNANT
Multiple Myeloma
• Multiple myeloma is a rare tumor of the bone marrow.
• Skull bone is affected and X-ray shows multiple, small,
translucent punched out areas in bones.
• The patient may have spontaneous fractures.
• Biochemistry shows raised globulin and Bence Jones
proteins in the urine.
• Bone marrow biopsy shows mitotic figures of myelocytes
and erythroblasts.
• Treatment is Chemotherapy (melphalan-drug of choice) or
Radiotherapy.

- MALIGNANT
Leukemia
• Twenty percent of leukemia patients eventually involve
ear/temporal bone.
• „They present as mucosal ulceration or bleeding from EAC.
• „The tympanic membrane and mucosa of middle ear
become irregular and thick.
• „The disease may eventually involve facial and auditory
nerves.

- MALIGNANT
Secondary Tumours
• Tumours of E.A.C, parotid gland, or nasopharynx
may invade middle ear cleft either through the
preformed pathways or bone erosion.
• Sometimes middle ear is the site of metastasis in
advanced cases of ca breast, bronchus, prostate,
kidney or G.I tract.

Tumours of External Auditory Canal and Middle Ear

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