Temporal Bone Neoplasm

Benign Tumors

Exostosis Osteoma

Cholesteatoma Keratosis Obturans

CPA tumor
Malignant Tumors

SCCA BCCA

Broad based lesion Multiple Lesions Cortex intact Exostosis

Exostosis
Location Frequently bilateral Along TS and TM suture lines
Arises near the annulus Broad based lesion, Multiple Lesions

Radiographic appearance Broad base Cortex intact Other Associated with prolonged cold water exposure

Single Lesion Pedunculated Unilateral No cortical invasion Osteoma

Osteoma in the external auditory canal is an uncommon benign lesion,

which presents as a solitary, unilateral, and slowgrowing pedunculated mass in the outer half of the bony canal. It is usually asymptomatic;
but symptoms can arise if a canal obstruction occurs.

Keratosis Obturans
is accumulation of desquamated keratin in the external auditory meatus.

Pathology:
The keratin plug seen in keratosis obturans appears like a geometrically patterned keratin plug within the lumen of expanded ear canal. These keratin squames are shed from the complete circumference of the deep ear canal forming a lamina . It appears like onion skin.

Etiology:

occur due to abnormal epithelial migration of ear canal skin.

The movement of the surface epithelium appears to be reversed in these patients.

(The surface epithelium over pars flaccida migrates downwards to the pars tensa and then moves inferiorly across the drum).

Types of keratosis obturans:

a. Inflammatory type:

due to acute inflammation involving the external ear canal. Viral infections commonly cause this problem. The inflammatory reaction involving the ear canal temporarily alters epithelial migration. This condition can only be cured by removal

b. Silent type:

no predisposing acute infections involved. This condition is postulated to be caused by abnormal separation keratin that persists even after the removal, and will need repeated removals.

c. Primary auditory canal cholesteatoma:

Etiology is uncertain. thought to be caused by trauma to the bone covering the external canal.

The piece of exposed bone in the external canal becomes infected and sequests.
The lining epithelium migrates into this area causing the formation of cholesteatoma.

Keratosis obturans commonly occur in young patients.

Clinical features:
1. Severe ear pain 2. Mild / moderate conductive hearing loss 3. Associated bronchitis / sinusitis - common

On examination:

The ear canal appears to be widened, making the ear drum stand out. CT scan of temporal bones may reveal canal erosion and widening.

Management:

1. Surgical removal under G.A. 2. Canal plasty is helpful in recurrent cases 3. Mastoidectomy should be performed in cases with primary cholesteatoma of external canal.

Cerebellopontine Angle

Area of the lateral (quadrimenal) cistern containing CSF, arachnoid tissue, cranial nerves and their associated vessels. Borders

Medial lateral surface of the brainstem Lateral petrous bone Superior middle cerebellar peduncle & cerebellum Inferior arachnoid tissue of lower cranial nerves Posterior cerbellar peduncle

Acoustic Neuroma

Comprises 60-92% of CPA lesions Majority of cases (95%) are sporatic Occur with equal frequency on the Superior and Inferior vestibular nerves Pathophysiology

Composed of Antoni A&B tissue Antoni A compact tissue with spindle cells in palisades (most common) Antoni B loose tissue with cyst formation.

An acoustic neuroma is a benign tumor on the vestibulocochlear nerve (eighth cranial nerve).

Alternative names:
Vestibular schwannoma .

Tumor acoustic .
Cerebellopontine angle tumor . Angle tumor . acoustic neurinoma.

The vestibular nerve, like many nerves, is surrounded by a cover called a myelin sheath which is formed by a schwann cell. A tumor sometimes develops from Overproduction of the myelin sheath of the Schwann cells, called a schwannoma

Acoustic neuroma can occur anywhere along the vestibular nerve but are most likely to occur where the vestibulocochlear nerve passes through the internal auditory canal.

stage I: it's called intracanalicular tumor and measured in millimeters. It slowly fill the IAC...

In Stage II: a "small acoustic less than 1.5 cm and called cisternal tumor protrude through the opening of the IAC on the brain side, into the Cerebellopontine angle (CPA).

In Stage III: A "moderate" acoustic about 1.5 to 3 cm called compressive tumor has grown to come into contact with the cerebellum and/or the brainstem, without compressing these brain structures...

Finally in Stage IV: if untreated a "large" acoustic about 3 cm or greater will deform the cerebellum and/or the brainstem...

As the acoustic neuroma grows it can affect:

The vestibular nerve causing vertigo and balance

difficulties (disequilibrium)

The cochlear nerve causing hearing loss and tinnitus

in the affected ear

As the acoustic neuroma grows it can affect:

The surrounding cranial nerves. i. 5th CN results in facial pain and or

numbness. ii. 6th CN causing double vision iii. 7th CN causing spasms, weakness or paralysis of the facial muscles. iv. 9th, 10th, or 12th CN resulting in swallowing and/or speaking difficulties.

As the acoustic neuroma grows it can affect:

If left untreated, the

tumor can become large enough to press against and affect the functioning of the brain stem Causing:
i. ii. iii. iv.

v. Sleepiness. vi. Coma. vii. Respiratory

difficulties viii. Death.

Headaches. Gait ataxia Tremors Nausea or vomiting

Acoustic neuroma occurs in two forms:

1. A sporadic form which account for

95% of all cases. The cause of the sporadic form is unclear.

2. A form associated with an inherited

syndrome called neurofibromatosis type II (NF2). Roughly 5% of patients with acoustic neuroma have NF2.

TYPE
Unilateral acoustic neurinomas:
Account for 8 percent of all tumors inside

the skull.

May develop at any age, but most often

occur between the ages of 30 and 60. by environmental factors.

May be the result of gene damage caused Most unilateral acoustic neuromas result

when the genes, that are responsible for the prevention of tumors, become spontaneously changed or missing.

Bilateral acoustic neurinomas

Develop in the teens or early

adulthood. Are hereditary, caused by neurofibromatosis-2 (NF2)

Diagnosis
Physical examination

The health care provider may diagnose an acoustic neuroma based on the history, neurological examination or testing of the patient .

Second: Pure tone and speech audiometry

Third: auditory brainstem response (ABR)

Fourth: Magnetic resonance imaging (MRI)

Other useful tests used to diagnose acoustic neuroma and to differentiate it from other causes of dizziness or vertigo include:

CT scan caloric stimulation

Electronystagmography

Treatment
There are five distinct treatment options:
Medical treatment or observation Surgery radiation Gamma-knife procedure Cochlear implantation

Paragangliomas

benign, slow growing tumors distributed throughout the head and neck can also be found in the orbit, the larynx, and along the course of the vagus nerve

Paragangliomas

various terminology used in past Glenner and Grimley divided the tumors into adrenal paragangliomas (or pheochromocytomas) and extraadrenal paragangliomas terms used in past include: glomus tumors, chemodectomas, carotid body tumors the correct terminology is paraganglioma based on the anatomical location

Pathology

all paragangliomas are closely related to each other and to pheochromocytomas of the adrenal gland histologic appearance is similar to normal paraganglia

paragangliomas consist of clusters of Type I or chief cells and Type II or sustentacular cells

Pathology

these clusters of cells make up the histologic structure termed Zellballen

nuclear pleomorphism and cellular hyperchromatism is common in paragangliomas

malignancy cannot be determined histologically but is reserved for presence of local, regional, or distant metastasis

Jugulotympanic Paragangliomas

second most common temporal bone tumor (after acoustic neuroma)

incidence of 1:1,300,000
female:male ratio 4:1

median age 50-60 yrs (range 6 mo - 88 yrs)

no ethnic or racial predilection

Jugulotympanic Paragangliomas

consist of sporadic and familial forms familial form has higher incidence of multicentricity incidence of secreting JTP is 1-3%

Jugulotympanic Paragangliomas

very slow growing spread locally in multidirectional fashion along paths of least resistance air cell tracts are most important route

can spread outside temporal bone via eustachian tube, vascular lumens, and neurovascular foramina including IAC

Jugulotympanic Paragangliomas bone erosion noted by crescentic lucencies

hypotympanum and carotid crest (separating ICA from IJV) are susceptible clinical course is slow asymptomatic growth until lesion far advanced

pt usually c/o pulsatile tinnitis. Other complaints may be aural fullness or HL

cranial nerve deficits, especially IX and X, can be seen with large tumors

Jugulotympanic Paragangliomas

otoscopic exam can be normal, can reveal red mass behind TM, or show a vascular ear polyp which may bleed spontaneously Browns sign = blanching of ME mass with positive pneumotoscopic pressure

Jugulotympanic Paragangliomas

just like carotid paragangliomas, a family hx should be sought

question pt regarding symptoms of secreting tumor(labile B/P, tachycardia, vascular HA)

any suspicion, obtain urine for VMA, circulating catecholamines if positive, get abdominal CT to r/o concomitant adrenal pheochromocytoma

Jugulotympanic Paragangliomas obtain audiogram

imaging should include CT temporal bone and MRI

on CT, JTPs show bone erosion around jugular bulb and carotid artery
CT helps delineate tumor relationship to facial n., cochlea, and ICA MRI helps evaluate intracranial extension, flow in ipsi and contralateral sigmoid, and further defines tumor relationship to ICA

Jugulotympanic Paragangliomas

arteriography is helpful if surgery is planned helps in detecting multicentric tumors, identifies feeding vessels, allows for embolization pre-op, identifies intrasinus and intravenous extension, provides further information on flow in contralateral sigmoid and internal jugular vein also allows for pre-op BTO if other images suggest extensive involvement of ICA

Jugulotympanic Paragangliomas Glasscock and Jacksons system divides JTPs into

glomus tympanicum and glomus jugulare tumors. Each group is subdivided based on size and extension

The Glasscock-Jackson and Fisch classifications of glomus tumors are widely used. The Fisch classification of glomus tumors is based on extension of the tumor to surrounding anatomic structures and is closely related to mortality and morbidity. Type A tumor - Tumor limited to the middle ear cleft (glomus tympanicum) Type B tumor - Tumor limited to the tympanomastoid area with no infralabyrinthine compartment involvement Type C tumor - Tumor involving the infralabyrinthine compartment of the temporal bone and extending into the petrous apex Type C1 tumor - Tumor with limited involvement of the vertical portion of the carotid canal Type C2 tumor - Tumor invading the vertical portion of the carotid canal Type C3 tumor - Tumor invasion of the horizontal portion of the carotid canal

Jugulotympanic Paragangliomas

JTPs are considered radiosensitive surgical goal is total or near-total removal RT goal is arrest of tumor growth much controversy over treatment of choice

Malignant Lesions
Location

May arise within EAC or extend from pinna, postauricular sulcus, or parotid Involvement or invasion of soft tissue with destruction of bony cortex Squamous cell CA Basal cell CA Salivary gland CAs

Radiographic appearance

Types

Malignant Lesions
Location

May arise within EAC or extend from pinna, postauricular sulcus, or parotid Involvement or invasion of soft tissue with destruction of bony cortex Squamous cell CA Basal cell CA Salivary gland CAs

Radiographic appearance

Types

SCC is most common malignancy of middle ear and temporal bone accounting for 60-80% of these lesion

BCC may b slightly more prevalent than SCC on the auricle but accounts for 20 % of neoplasm of the middle and temporal bone

Most often cancer of the temporal bone is caused by a tumor that has spread from the skin of the pinna (the external part of the ear). This is because the skin of the pinna undergoes years of sun exposure, which can lead to basal cell cancer or squamous call cancer development.

Basal Cell Carcinoma

the most common and the least aggressive type of skin cancer.
appears as a pearly white lesion that grows very slowly. Painless lump on the skin that does not cause any discomfort, at times however it can ulcerate, become painful, and bleed. very slow growth rate very low potential for spread to other parts; due to these special characteristics we are able to treat it with a much less radical surgical treatment (meaning the tumor does not need to be removed with a large rim of normal tissue to assure cure)

Squamous Cell Cancers

more aggressive than basal cell tumors and as such require a larger surgical excision (the tumor needs to be removed with a larger rim of normal tissue as compared to a basal cell cancer). more destructive, tends to grow deeper, and has a much higher potential to spread. These tumors need to be treated immediately. .

Symptoms and Diagnosis

On the external ear (Pinna), most often a scaly area of the skin appears that does not seem to resolve with any treatment/moisturization. This is usually the first sign of a skin cancer, followed by development of a mass, which appears as a bump
Tumors that grow inside the ear canal cause hearing loss, chronic discharge and at times bleeding .

Tumors that extend into the temporal bone cause more severe pain, hearing loss, and vertigo and Facial paralysis..

Benign Osteoma Neurofibroma Paraganglioma Adenoma

Schwannoma
Chordoma Hemangiopericytoma Lipoma

Malignant
Squamous cell carcinoma Basal cell carcinoma Adenocarcinoma Acinic cell carcinoma

Osteosarcoma
Chondrosarcoma Rhabdomyosarcoma (inchildren) Metastatic carcinoma Lymphoma Malignant neuroma Malignant paraganglioma CNS malignancy

Pathophysiology
The complex anatomy of the temporal bone makes tumor spread difficult to predict. Tumors of the skin around the auricle may extend along the soft tissues of the neck and ear. The fissures of Santorini, foramen of Huschke, and bonycartilaginous junctions are a source of direct access to the periparotid tissues and temporomandibular joint. Cancer in the external auditory meatus can invade posteriorly through the soft tissue into the retroauricular sulcus over the mastoid cortex.

. Tumor growth medially along the EAC can extend through the tympanic membrane and bony tympanic ring, allowing invasion into the middle ear

In the middle ear or mastoid, tumors spread easily via the eustachian tube, round and oval windows, neurovascular structures, and extensive air spaces of the mastoid cavity. Aggressive tumors can erode through the tegmen tympani or mastoid into the middle or posterior fossa. The sigmoid sinus may become involved. The facial nerve and the stylomastoid foramen are metastatic routes to the soft tissues of the neck and the parotid Nodal metastasis is uncommon in early disease but may occur in 10-20% of cases of advanced disease.

Etiology

to non melanomatous skin cancers A genetic predisposition to skin cancer Chronic otitis media and cholesteatoma are common in patients with temporal bone cancers

aged 60 years or older, although any age group, including children, can be affected.

Symptoms
Otalgia (80-85%) Otorrhea (40-75%) Facial paralysis (25%) Hearing loss (45-80%) Vertigo Auricular lesion External canal mass (10%) Parotid mass (19%) Skin lesions CN V, IX, I, XI deficits (30%)

Tinnitus (8-10%)

Investigation

Imaging Studies CT scanning of the temporal bone and neck: MRI with gadolinium enhancement can be helpful because it better delineates soft tissue interfaces.

Chest radiography: If the histology indicates squamous cell carcinoma, obtain plain radiographs or CT scans of the chest to rule out metastasis.

Carotid angiography with balloon occlusion Xenon test:

1. demonstrate the adequacy of cerebral blood flow from the

contralateral carotid artery.

2.

venous outflow phase to determine the adequacy of the contralateral sigmoid/jugular system in case the surgery

Other Tests Audiometry: An audiogram is obtained prior to performing any major procedure on the ear or temporal bone. Audiograms provide baseline hearing thresholds for future comparison.

Electrocardiography

Treatment
Indications In general, all patients who are medically able should undergo surgical treatment. Primary radiation is ineffective for curative treatment; however, in the most extreme cases in which contraindications to surgery are serious deterrents to surgery, palliative radiation and chemotherapy may be offered

Staging system proposed by Pittsburgh, lesions were defined as follows:[8]

T1 - Tumor limited to the EAC without bony erosion or evidence of soft tissue involvement

T2 - Tumor with limited EAC bone erosion (not full thickness) with limited (< 0.5 cm) soft tissue involvement
T3 - Tumor eroding the osseous EAC (full thickness) with limited (< 0.5 cm) soft tissue involvement or tumor involving the middle ear, mastoid, or both T4 - Tumor eroding the cochlea, petrous apex, medial wall of the middle ear, carotid canal, or jugular foramen of dura; or with extensive soft tissue involvement (>0.5 cm), such as involvement of the temporomandibular joint or stylomastoid foramen; or with evidence of facial paresis

Nodal involvement and stage can be classified as it is for other cancers of the head and neck.

N1 - Single ipsilateral lymph node, size less than 3 cm

N2 - Single ipsilateral node, size 3-6 cm N2b - Multiple ipsilateral nodes, all less than 6 cm N2c - Bilateral or contralateral nodes, all less than 6 cm N3 - Nodes involved greater than 6 cm

Cancer is staged as follows: Stage 0 - Tis N0 M0 Stage I - T1 N0 M0 Stage II - T2 N0 M0

Stage III - T3 N0 M0, T1 N1 M0, T2 N1 M0, T3 N1 M0

Stage IV - T4 N0 M0, T4 N1 M0, any T N2 M0, any T N3 M0, any T any N M1

Medical Therapy
Primary radiation is ineffective for curative treatment.

In the most extreme cases in which contraindications to surgery are serious deterrents to surgery, palliative radiation and chemotherapy may be offered.
Postoperative radiation treatment may be indicated in advanced disease. Most authors advocate full course postoperative radiation to stage T3 or T4 tumors as defined by the University of Pittsburgh staging system.

Surgical Therapy
The optimal surgery removes all of the cancer en bloc because positive margins are associated with poor survival rates. The resection procedures that can be performed for the temporal bone include a modified lateral temporal bone resection, lateral temporal bone resection, subtotal temporal bone resection, and total temporal bone resection. Adjunctive surgical procedures, including neck dissection, parotidectomy, and craniotomy, should be performed when indicated. A