squamous cell carcinoma of the temporal bone

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104 The Otorhinolaryngologist 2011; 3(3): 104-110 © Copyright 2011 Rila Publications Ltd. Abstract Squamous cell carcinoma is the most common malignancy of the ear canal and the temporal bone. It is a rare and aggressive disease that poses significant challenges to the clinician. In this review we describe the evaluation and management of those tumours. Keywords Squamous cell carcinoma, temporal bone, external ear, middle ear. Introduction Squamous cell carcinoma (SCC) of the external auditory canal (EAC) and temporal bone is a rare entity representing less than 1% of all head and neck malignancy, but it accounts for 90% of all malignant tumours of the temporal bone. 1, 2 These tumours pose major challenges to the clinician in terms of diagnosis, staging and management. Since it’s early clinical description by Wilde and Schwartze in 1775, and subsequent histopathological confirmation by Polizer, treatment approaches have greatly changed. 3, 4 In the early twentieth century, radical mastoidectomy followed by radiation was the standard treatment for SCC of the temporal bone. 5 Later, Campbell and colleagues raised the possibility of temporal bone resection but it was Parsons and Lewis who reported the first one-stage subtotal resection of the temporal bone in 1954. 6-8 Piecemeal, subtotal and extended temporal bone resections, including the removal of the parotid gland with or without selective neck dissections, followed by radiotherapy have all been described. 3, 8 This review aims to describe the current status in diagnosis, staging and management of this challenging disease. The incidence of malignancy is higher in women at approximately 1 in 1000 000 of population per year, and for men 0.8 per1000 000 of population per year. 9 Though it affects all ages, the peak incidence is in the seventh decade. 10-12 It is important to remember that parotid malignancies and skin malignancy of the conchal bowl spreading medially to involve the EAC and temporal bone are far more common than primary cancers of the temporal bone. Anatomy Temporal bone SCC often presents late. Both the middle ear cleft and EAC are usually involved at the time of presentation making it difficult to determine from where it originated. 12-14 Once established the tumour usually spreads by direct invasion and extension. 15 The anatomy of the temporal bone provides an insight in to the routes of spread (Figure 1): 16 N Sethi, K Michalopoulos, Y Bajaj, S Sood & M I Khan * N Sethi * K Michalopoulos * Y Bajaj * S Sood * M I Khan * Bradford Teaching Hospitals NHS Trust Correspondence: Mr M I Khan Consultant ENT Surgeon Department of Otolaryngology Bradford Royal Infirmary Duckworth Lane, Bradford BD9 6RJ UK Reviews Figure 1. Routes of spread via direct invasion. 16 Squamous Cell Carcinoma of the Temporal Bone The Otorhinolar V3 N3.qxd:The Otorhinolar V3 N3 4/26/2011 8:13 PM Page 104

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104 The Otorhinolaryngologist 2011; 3(3): 104-110

© Copyright 2011 Rila Publications Ltd.

AbstractSquamous cell carcinoma is the most common malignancy ofthe ear canal and the temporal bone. It is a rare andaggressive disease that poses significant challenges to theclinician. In this review we describe the evaluation andmanagement of those tumours.

KeywordsSquamous cell carcinoma, temporal bone, external ear,middle ear.

IntroductionSquamous cell carcinoma (SCC) of the externalauditory canal (EAC) and temporal bone is a rareentity representing less than 1% of all head and neckmalignancy, but it accounts for 90% of all malignanttumours of the temporal bone.1, 2 These tumourspose major challenges to the clinician in terms ofdiagnosis, staging and management. Since it’s earlyclinical description by Wilde and Schwartze in1775, and subsequent histopathologicalconfirmation by Polizer, treatment approaches havegreatly changed.3, 4

In the early twentieth century, radicalmastoidectomy followed by radiation was thestandard treatment for SCC of the temporal bone.5

Later, Campbell and colleagues raised the possibilityof temporal bone resection but it was Parsons and

Lewis who reported the first one-stage subtotalresection of the temporal bone in 1954.6-8

Piecemeal, subtotal and extended temporal boneresections, including the removal of the parotidgland with or without selective neck dissections,followed by radiotherapy have all been described.3, 8

This review aims to describe the current status indiagnosis, staging and management of thischallenging disease.

The incidence of malignancy is higher in womenat approximately 1 in 1000 000 of population peryear, and for men 0.8 per1000 000 of population peryear.9 Though it affects all ages, the peak incidence isin the seventh decade.10-12 It is important toremember that parotid malignancies and skinmalignancy of the conchal bowl spreading mediallyto involve the EAC and temporal bone are far morecommon than primary cancers of the temporal bone.

AnatomyTemporal bone SCC often presents late. Both themiddle ear cleft and EAC are usually involved at thetime of presentation making it difficult to determinefrom where it originated.12-14 Once established the tumour usually spreads by direct invasion andextension.15 The anatomy of the temporal boneprovides an insight in to the routes of spread (Figure 1):16

N Sethi, K Michalopoulos, Y Bajaj, S Sood & M I Khan

*N Sethi

*K Michalopoulos

*Y Bajaj

*S Sood

*M I Khan

*Bradford TeachingHospitals NHS Trust

Correspondence:Mr M I KhanConsultant ENT SurgeonDepartment ofOtolaryngologyBradford Royal InfirmaryDuckworth Lane, Bradford BD9 6RJUK

Reviews

Figure 1. Routes of spread via direct invasion.16

Squamous Cell Carcinoma of the Temporal Bone

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1. Anteriorly through the cartilaginous canal (fissures ofSantorini) into the parotid gland, infratemporal fossaand down into the temporomandibular joint. Thiscauses trismus, pain and preauricular swelling.

2. Through the concha into the post-auricular sulcus.3. Inferiorly into the stylomastoid foramen and fallopian

canal resulting in a facial nerve weakness. If the jugularforamen is involved, dysphagia and dysphonia can occur.

4. Medially through the tympanic membrane andtympanic cavity into the middle ear, causingconductive hearing loss, facial palsy.

5. Into the anterior mesotympanum, into the Eustachiantube and carotid sheath.

6. Into the inner ear through the round window causingvertigo and sensorineural hearing loss.

7. Along the extratemporal facial nerve into theinfratemporal fossa.

8. Inferomedially into the jugular fossa, carotid artery andlower cranial nerves.

The lymphatic drainage of the EAC follows that of theauricle and includes the parotid lymph glands anteriorly,the mastoid lymph glands posteriorly, and thejugulodigastric nodes and the nodes overlying thesternomastoid muscle inferiorly.14 Local lymph nodeinvolvement is less common though not rare occurring in10% to 20% of cases.14, 17 The rate of both regional anddistant metastases is also low ranging from 9 to 18%.18

AetiologyTo date, the exact cause of temporal bone carcinomaremains unclear. Although a number of risk factors havebeen proposed, they are difficult to assess due to the rarityof the disease. The presence of chronic suppurative otitismedia and SCC of the temporal bone has been frequentlyreported and is found in up to 60% of patients.4, 19

External beam radiotherapy is a well-recognised riskfactor for temporal bone carcinoma. This is particularlynoticeable in Asian populations where nasopharyngealcarcinoma is common and radiotherapy a commontreatment modality.19, 20 These have been reported withlong latency periods of up to 15 years.20

Human papilloma virus has been isolated in temporalbone SCC (as in other areas of head and neck SCC)though it’s exact role here is not clear.21 Cholesteatoma,chlorinated disinfectants and ultraviolet light remainreported but of unlikely significance.22, 23

Clinical features (Figure 2)The most common presenting symptoms include offensiveotorrhoea, otalgia and bleeding.8, 11, 14 The symptomsclosely resemble those of chronic otitis media and this

often leads to a delayed diagnosis or an unexpected one atelective mastoid exploration.17 Other presentingsymptoms can include hearing loss, facial palsy and pre-auricular masses. The possibility of carcinoma must alwaysbe considered when there is concern over necrotising otitisexterna and multiple biopsies should be performed.10, 24 Acomparison of the proportions of common presentingsymptoms from case series is shown in Table 1.

Examination of the ear can reveal an exophytic orpolypoidal mass, but may show only an ulcerated orgranular area.3 Any suspect lesions should be examinedmicroscopically, debrided and biopsied for pathologicalexamination. Cranial nerve palsies are a late sign of tumourspread. 28 The most commonly affected is the facial nerveleading to facial spasms and palsies, but theglossopharyngeal and vagus nerves can also be affected.28, 29

As such, a full neurological examination is necessary in allpatients suspected of having a malignancy as well asnasendoscopy to assess the nasopharynx and vocal cords.3, 12

Cervical lymphadenopathy must be assessed as this hasmajor implications for the patient’s prognosis.10

Diagnosis and InvestigationsAs with any tumour, histological confirmation of theprimary lesion is the gold standard for diagnosis. Asmentioned earlier the vast majority of tumours in thetemporal bone are SCC. Other types include basal cellcarcinoma, adenocarcinoma, melanoma, pseudo-epithelialhyperplasia and metastatic disease.10 Multiple, deepbiopsies must be taken due to the presences of secondaryinfection and oedema. If these are negative and clinicalsuspicion remains then repeat biopsies must beperformed.10 The use of CT-guided fine needle aspirationhas been reported to obtain a tissue diagnosis.30 If any

© Copyright 2011 Rila Publications Ltd.

Figure 2. Squamous cell carcinoma of the External Auditory Canal.

Symptom Moffat et al17 Nyrop et al11 Zhang et al25 Yin et al26 Leonetti et al27

Otorrhoea 69 45 78 42 69

Otalgia 64 35 45 24 73

Hearing loss - 25 30 2 69

Facial Palsy 17 - 36 4 30

Table 1. Comparison of percentage of common presenting symptoms.

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palpable neck nodes are found these should also beinvestigated with fine needle aspiration to aid staging andtreatment planning.

ImagingHigh resolution computed tomography (CT) andmagnetic resonance imaging (MRI) scans are combined intemporal bone SCC to provide information about the localextent and to stage the disease. CT scanning has been longestablished as the initial investigation of choice as it is a reliabledemonstrator of bony erosions of the external auditory canal,which is often the first sign of local spread of the disease.12, 31,32 It is also used preoperatively to assess the stage of disease andthere is good correlation between preoperative CT scaninterpretations and histological findings.12, 32

Pitfalls of CT include: difficulty in differentiating f luidand inf lamed mucosa from tumour in the middle ear ormastoid; distinguishing inf lammation from tumour wherethere is no adjacent bone erosion and determining theanterior soft tissue extent of the tumour.12, 16, 32 These canresult in underestimation of the disease in theinfratemporal fossa, the mastoid cavity and the carotidcanal.27

Magnetic resonance imaging (MRI) complementsCT scanning by offering additional information on the

soft tissue extent of the tumour.12 Infratemporal spread,perineural disease, dural and brain involvement can beseen clearly on MR scans.33, 34

Arteriography can be considered if the CT or MRIsuggests that the internal carotid artery is involved.10

Balloon occlusion can be performed to assess cross-circulation from the circle of Willis. If balloon testing ispassed the internal carotid can be permanently occludedwith coils prior to surgical resection.35, 36

Audiometry is mandatory for both the involved andcontra lateral ear to identify pre-existing deficits (ashearing loss is unavoidable with treatment) and forsubsequent auditory rehabilitation.10

StagingStaging has evolved through various systems. A summaryof these is presented in Table 2.

Arriaga et al devised the University of Pittsburghstaging system in 1990 based on preoperative clinicalexamination and CT findings. It conforms to theAmerican Joint Cancer Committee’s TNM classificationsystem and is limited to a single tumour type (SCC).37 Thisallows for preoperative staging, planning of treatment andevaluation of outcomes. This has been utilised in severalcase series to good effect and validation.12, 16, 38 More

© Copyright 2011 Rila Publications Ltd.

Author Staging system

Stell & McCormick40 T1 limited to site of originT2 extends beyond site of origin, but not organ of originT3 extends to dura, skull base, parotid, temporomandibular joint (TMJ)

Kinney41 1 Limited to EAC2 Bony erosion or invasion of ME3 Extension to dura, skull base, stylomastoid foramen

Shih & Crabtree42 1 Localised to EAC2 Extends to temporal bone3 Extends to parotid, neck, skull base, dura

Spector43 1 Limited to EAC2 Superficial invasion3 Deep invasion4 Extends beyond temporal bone

Pensak et al28 I single site tumour <1cmII single site tumour >1cmIII transannular extensionIV mastoid/petrous invasionV periauricular/contiguous extensionVI neck nodes, distant site or infratemporal fossa

Manolidis et al44 1 Confined to EAC2 Involving TMJ, parotid or infratemporal fossa3 Spread to ME, mastoid, facial nerve4 Spread to dura, jugular bulb, sigmoid sinus, ICA or petrous apex

Modified Pittsburgh system39 T1 Limited to EACT2 limited bony erosion or limited (<0.5cm) soft tissue involvementT3 Full thickness bony erosions with limited soft tissue involvement, or involving ME and/or mastoidor causing facial palsy at presentationT4 Eroding the cochlea, petrous apex, medial wall of ME, carotid canal, jugular foramen, dura orwith extensive (>0.5cm) soft tissue involvement

Table 2. Summary of staging systems.

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recently Hirsh modified the Pittsburgh staging system toupgrade the impact of facial nerve involvement to thedefining feature of a T3 tumour.39 These revisions have ledto this being the current gold standard for staging temporalbone malignancy.

ManagementTemporal bone malignancies should always be managed inthe setting of a multidisciplinary team. The options aresurgery, radiotherapy (with or without chemotherapy) or acombination of these two modalities. The best results areobtained with combined surgery and post-operativeradiotherapy.3, 13

Distant metastases are uncommon.2, 16, 26 This mayindicate that local aggressive invasion may be the mainbehaviour for temporal bone carcinomas rather thandistant metastasis. Certainly local failure remains the mainproblem with these patients.45

SurgeryThe surgical treatment for carcinoma of the temporal boneincludes:

Management of the primary siteManagement of the neckReconstruction

Management of the primary siteThe type of resection is determined by the stage of thetumour. It is recommended that excision be as close to anen-bloc resection as possible rather than piecemeal orsleeve resections.10, 46, 47

Lateral Temporal Bone Resection (LTBR) For T1 and T2 lesions a lateral temporal bone resection isthe minimum that should be performed.3 The pinna,EAC and tympanic membrane are resected en bloc withthe superficial lobe of the parotid (Figure 3). This isachieved by performing an extended corticalmastoidectomy. The facial recess is opened via posteriortympanotomy and dissection extended inferiorly,anterior to the facial nerve. The entire bone lateral to thefacial nerve, jugular fossa and carotid canal is dissected.Dissection is continued anteriorly into the root of thezygoma and the temporomadibular joint. The

incudostapedial joint is separated and, with gentlepressure, the anterior wall of the middle ear is fracturedfor en bloc removal of the EAC. The posterior aspect ofthe TMJ is the anterior margin of the tumour. Moffatendorses removing at least the articular disc and glenoidfossa due to difficulty assessing any bony erosion of thethin anterior wall of the EAC. The Eustachian tube isobliterated and the middle ear mucosa is excised to createa bed for the f lap repair.3, 10, 46

Extended temporal bone resection (ETBR) Tumours extending beyond the EAC (T3 and T4) requirea more extensive resection. If tumour involves the middleear or mastoid (T3), a subtotal resection is required. Thisincludes LTBR with additional removal of the oticcapsule. The dissection can be extended further mediallyto the petrous apex and skull base to follow the extent ofthe disease. This is usually achieved with piecemealdissection and, in most cases, the facial nerve would besacrificed.

The entire temporal bone lateral to the petrous apex isremoved. The carotid artery is delineated anteriorly, andthe dissection is completed between middle fossa durasuperiorly, posterior fossa dura and sigmoid sinusposteriorly and jugular bulb inferiorly.

For more extensive tumours (T4) the dissection maybe extended further with the options of petrous apexresection (total temporal bone resection), craniotomies,and mandibulectomy and parapharyngeal/infratemporalfossa resection. Intracranial resections may necessitateresection of dura or brain, sacrificing the internal carotidartery and lower cranial nerves.3, 17

Grafting of the facial nerve should be performed at thetime of primary surgery, if possible, using donor nervessuch as the greater auricular nerve, sural nerve or lateralcutaneous nerve of thigh.

Parotidectomy has become a component of the surgicaltreatment of carcinoma of the temporal bone as the parotidforms the anterior margin of the tumour and theintraparotid nodes are the first echelon nodes drainingexternal auditory canal. Superficial parotidectomy isperformed for T1 & T2 tumours and total parotidectomyfor T3 & T4 tumours.3, 10, 17

If dura and brain are involved with the tumour, it isexcised as a separate specimen with frozen sectionmicroscopy of all margins. The remaining defect is thenrepaired with fascia lata.17

Management of the neckNeck nodes at presentation have been reported in 10-23% ofcases.3, 4, 40 Positive nodal involvement indicates an aggressivenature of the tumour and is a poor prognostic indicator.4

Patients who are clinically and radiologically nodenegative should have a selective neck dissection of levels I-III. Node positive patients require a modified radical neckdissection. This helps with staging and further decisionsregarding the need for post operative radiotherapy to theneck.3, 17 The majority of patients will require a neckdissection regardless as most will undergo free f lapreconstruction following tumour resection.

© Copyright 2011 Rila Publications Ltd.

Figure 3. Resected specimen.

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Reconstruction The operated area after excision usually leaves a sizeable defect(Figure 4). The gold standard in modern reconstruction isfree tissue transfer.10 Netterville described five criteria tomaximise chances of a successful reconstruction:48

1. adequate coverage of vital structures2. competent dural closure to prevent cerebrospinal

(CSF) leak3. cosmetically acceptable reconstruction4. aeration of the middle ear if appropriate5. simplicity

The anterolateral thigh free f lap has become apopular choice for primary reconstruction offering bulk,skin and the ability to be harvested concomitantly whilsttumour resection is being performed.49, 50 Alternativedonor sites for free f laps include the radial forearm,rectus abdominis, deep inferior epigastric artery orlateral arm. Pedicled myocutaneous f laps, such astrapezius f lap, can be used if microvascularreconstruction is not possible. Temporalis f laps can beused for small volume defects only.

ComplicationsCranial nerve palsiesThe facial nerve is sacrificed in ETBR and is at risk ofdamage during LTBR. Corneal protection is vital in theevent of facial palsy as is rehabilitation to help withfunctional and cosmetic defects.3, 10 Glossopharyngeal,vagal and/or hypoglossal palsies can cause problems withswallowing and place the patient at risk of aspirationpneumonia and malnutrition. Involvement from speechand language therapy and dietitians is very important andfeeding alternative such as nasogastric tubes orgastrostomies may be needed.3

Flap failureFlap management is a complex challenging area and onewhich can be a cause of significant morbidity to thepatient. Flap dehiscence, f lap failure and haematomas arethe main complications requiring revision or salvageprocedures.50

CSF leakThis is a potential complication when the dura is breachedand can be minimised by good watertight dural repair andpostoperative lumbar drain insertion.3, 10, 17

Adjuvant Therapy

RadiotherapyAlmost all patients should receive postoperative radicalradiotherapy (60 Gy) to both the primary site andipsilateral neck, irrespective of the type of surgery.8 Ifsurgery is not possible primary radiotherapy can beadministered. Radiotherapy has been used as a singletreatment modality in patients with early disease to goodeffect.26

ChemotherapySynchronous chemotherapy has been shown to enhancethe effects of radiotherapy.26 This involves cisplatin,however the side effects of chemotherapy are also increasedwhen used in conjunction with radiotherapy. Thisincludes ototoxicity and brain toxicity.51 The epidermalgrowth factor antagonist, cetuximab, has also been shownto enhance the effect of radiotherapy without a substantialincrease in the side effects.52

PrognosisSurvival is intrinsically related to the stage of the disease. InStage I and II survival rates are reported as high as 80-100%.2, 8, 16, 26 However survival decreases with increasedsize of primary tumour and the presence of nodal ormetastatic disease. This comes down to around 50% forstage III and ranges from 7 – 50% for stage IV.8, 11, 16,38

Moffat et al found that the single most importantprognostic factor was lymph node involvement.8

Unsurprisingly the survival rates in patients withinvolved resection margins are significantly lower thanthose with free margins and as such Gillespie advocates theuse of frozen section microscopy to examine marginsintraoperatively.12, 26

Tumour differentiation also has an impact onoutcome, and as with other cancers, the lesser thedifferentiation the worse the outcome.3, 10

Dural and cerebral involvements are poorprognosticators, and for some render the tumourinoperable, though of course it adds to the patient’s alreadyhigh risk of morbidity.3, 12

The intrapetrous carotid artery is relatively resistant totumour invasion. Therefore involvement signif iesextensive disease and confers a worse prognosis.8, 12

Reports of small series of radiation-associated tumourshave suggested that these patients have a worse prognosiswith poorer overall survival.19, 53

SummaryCarcinoma of the temporal bone is a difficult disease tocure. Debate continues over the best staging system andindeed over the best modality for managing the disease.For anything other than very early limited disease the beststrategy appears to be an en-bloc style resection (eitherLTBR or ETBR) with excision of the pinna, TMJ andparotid as appropriate.8, 17

© Copyright 2011 Rila Publications Ltd.

Figure 4. Resection completed; reconstruction now required withfree flap.

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All patients are recommended to have at least a levelI - III neck dissection for staging. This also helps withfacilitating free f lap reconstruction as well as decisionsregarding post-operative radiotherapy.8, 10

Poor prognostic factors include positive neck nodes,poorly differentiated histology, dural, brain or carotid

involvement and positive excision margins. Early diagnosisand aggressive primary surgical treatment followed bypostoperative radiotherapy offer the best chance of cure forthe patient.

© Copyright 2011 Rila Publications Ltd.

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