Tumors of external and middle ear

1. Dr.Ramesh Parajuli, MS
Chitwan Medical College Teaching Hospital,Bharatpur-10, Chitwan, Nepal

2. Contents
• Introduction
• Tumours of auricle
• Benign tumours of external auditory canal (EAC) & middle
ear cleft
• Malignant tumours of external auditory canal (EAC) &
middle ear cleft

3. Introduction
• Less common
• Little known
• Some surgeons - never encounter
• Difficult to treat
• Outcome: unsatisfactory

4. Tumours of auricle

5. Basal cell & Squamous cell carcinoma
Epidemiology:
• Mean age 70 yr
• Lower in darker-skinned ethnic group
• Basal cell (BCC)>Squamous (SCC)>Melanoma (MM)
• 85-95% of all BCC & SCC occur in head & neck region
• 12% of these tumours in auricle
• SCC: External ear & upper face
• BCC: Midface & auricle

6. Pathophysiology/Risk factors
1. Sun exposure: ultraviolet radiation (SCC> BCC)
2. Actinic keratosis: small, scaly lesions, common
(Actinic keratosis60% SCC)
3. Keratoacanthoma: benign conditionspontaneous resolution or
SCC
4. Exposure to radiation
5. Immunosupression: 5-16 fold

7. 7. Xeroderma pigmentosa: Autosomal recessive, DNA repair
mechanism, SCC and BCC at young age
8. Trauma
9. Frostbite
10. Psoriasis
11.Aflatoxin B
12. Nevoid basal cell syndrome (Gorlin’s syndrome): Autosomal
dominant, multiple pigmented BCC once reach puberty
13. Chronic otitis externa

8. Pathology
Basal Cell Carcinoma (BCC)
Variants of BCC:
 Nodular: most common, least aggressive,
bleeds easily
 Ulcerative: “Rodent ulcer”
 Pigmented:
 Superficial:
 Morpheaform/sclerosing: Most aggressive,
highest rate of recurrence
 Basaloid squamous

9. Squamous cell carcinoma (SCC)
• Plaque to nodule or ulcer
• Variants of SCC, less common than BCC
• Keratin pearl
• Variants of SCC:
 Nodular
 Ulcerative
 Pigmented: confused with melanoma
 Spindle shaped subtype: radiated skin
 Verrucous- locally destructive
 Basaloid squamous
 Adenoid squamous

10. Diagnosis
• Clinical evaluation
• Biopsy:
• Lesion with change in colour, size, shape,
friable or ulcerated
Punch biopsy- preserves architecture of the
lesion for histologic analysis, depth of
lesion, excision with precise margin.
• Imaging studies (CT, MRI): rarely needed

11. Staging of Tumour (AJCC)

12. Stage Primary
tumour
Regional
lymph
node
Distant
metastasis
Stage 0 Tis N0 M 0
Stage I T1 N0 M 0
Stage II T 2 N 0 M 0
T 3 N 0 M 0
Stage III T 4 N 0 M 0
Any T N 1 M 0
Stage IV Any T Any N M 1

13. Limitations of AJCC staging in malignancy of ear
 Thin skin of ear early involvement of deeper structure T4
T4- doesn’t have similar prognosis or require radical treatment
like in other sites
 Staging based on sizeless practical (unique anatomy of ear)
 Even small tumours eg. preauricular, concha or
tragusextensive surgery
 Doesn’t account for different histologies: BCC may act
differently than SCC

14. Treatment
Depends on
• Location & size
• General condition of patient
• Expertise of doctor
• Desire of patient

15. Surgical Excision
• Most common form of treatment
• Appropriate margin of resection difficult
• BCC:
– 8 mm for <3 cm
– 1.5 cm for >3cm (Bumstead et al., 1981)
• 2-3 mm for <1cm
• 3-5mm for 1-2 cm
• 7-10mm-morpheaform histology (Scotto et al., 1983)
• SCC:
1-2 cm surgical margin (Bumstead et al., 1981)

16. Mohs’ Surgery
Dr. Frederick Mohs in 1941
• Serial horizontal sectioning of tumor & surrounding tissue with
immediate microscopic analysisconfirm the margins clear of
tumour
• Fixed in vivo with zinc chloride
• Several advantages over traditional wide local excision
 Horizontal section of entire margin analysis of small islands of
tumor cells
 Avoids unnecessary excision of normal tissue
 Less recurrences following Mohs’ surgery

17. • Recommended method for
 Malignancy arising in vital area
 Recurrent or previously treated area
 Aggressive histopathology
 Larger carcinoma >1cm
 Poorly defined margin

18. • Mohs’ surgery: overall cure rates for auricular carcinoma- 98% for
BCC and 92% for SCC (Mohs F, 1998 and Niparko et al., 1990)

19. Other surgical techniques:
For small non-agressive tumours with clearly
defined margin
• Curettage and electrodesiccation
• Cryosurgery: -40°c, cure rate exceeds 95%
Cryogun used to
spray liquid nitrogen

20. 5 year recurrence rate for primary BCC
(Rowe et al., 1989)

21. 5 year recurrence rates for previously treated BCC
(Rowe et al., 1989)

22. Radiation Therapy
• Advantages
 Avoids tissue defects, unfit patients, refuse surgery & tumour
involving adjacent areas
• Disadvantages
 Multiple treatments, more costly, risk of radiation induced tumour
• Regimen vary from center to center
• Common regimen
– 20 Gy, 2# very small tumor
– 30-40 Gy for 2-4 cm volume
– 60-65 Gy for large tumors
• Cure rate comparable to surgery for <1 cm

23. • Non surgical options
– Topical 5-Fluoruracil (5-20% concentration)
– Intralesional interferon-alpha
– Use of photodynamic therapy
• Treatment of recurrent disease- more difficult
Aggressive tumours, margins less precise and disrupted
anatomy due to prior treatmentreconstruction more difficult
• Treatment of metastatic neck disease
– Overall recurrence rate for BCC- <5% and SCC- 10 to 15%
– Elective neck dissection - Controversy

24. Reconstruction of auricle:

28. Malignant melanoma
Epidemiology:
• Incidence increasing
• Accounts for majority of deaths from skin malignancies
• 7-13% of all head & neck malignancy, 1% of all melanoma
• 65-79 yr
• Lower in black & dark skinned people
• Lower in woman- more attention paid on appearance & more
regular physician visits

29. Pathophysiology
• Sun exposure:
Acute severe & cumulative
• Actinic precursor
• Congenital naevi
• Familial dysplastic naevus syndrome
• Xeroderma pigmentosa
• Immunosuppression
Dysplastic nevus

30. Types of Melanoma
• Cutaneous melanoma
 Acral lentiginous
 Superfical spreading melanoma- most common
 Lentigo maligna melanoma
 Nodular melanoma
 Amelanotic melanoma
• Mucosal melanoma
Acral lentiginous
Nodular melanoma

31. Diagnosis
• Keen eyes, diligent physical examination
• Majority arises from pre-existing naevi
• A B C D (Asymmetry, Border, Color, Diameter)
• Excisional biopsy
• HPE: Variable
• Immunohistochemistry: S-100, Vimentin, HMB-
45
• Role of imaging

34. Treatment
Surgery
• Tumour thickness- correlates with survival
• 1cm for 1mm thickness & 2cm for larger
• Mohs’ Surgery: Controversy
• Overall cure rate: 68%
• Recurrence between 1-3 yr, follow up every 1-2 month
• Role of radiation
• Traditionally considered to be radioresistant
• For local control of paritally resected melanoma

35. Treatment of neck metastasis
• 17% nodal disease at presentation
• Auricular melanoma risk 42%
• Parotid & upper jugular digastric
• Radical or modified neck dissection for melanoma- No studies
to demonstrate differences
• Post-operative RT
• Elective neck dissection: Controversy
• Treatment of advanced disease: 10% 10 yr survival

36. Other tumours
• Merkel cell carcinoma:
 One of the most aggressive
 High recurrence and metastases rate
 Neuroendocrine tumour
 Pleuripotent basal cells
 Rapidly growing, firm, nodular
 Wide local excision
 Radiosensitive

37. • Malignant fibrous histiocytoma
• Dermatofibrosarcoma
• Angiosarcoma
• Metastatic disease from other sites

38. Temporal bone neoplasms

39. Contd…
• All three primordial layers  temporal bone spectrum of
neoplasms
• Benign & malignant
• Neither AJCC nor the UICCsystem for classifying temporal
bone tumours
• Difference in growth rate & sites of origin varied
presentations

40. Classification of tumours of the temporal bone:

41. Benign tumours of EAC and middle ear cleft
Exostosis
• Benign
• Deep part of bony canal, adjacent to TM
• Smooth, sessile, multiple, inner part of
meatus & bilateral
• Relationship with cold water exposure
• Asymptomatic
• >80% stenosis: Symptomatic

42. • Diagnosis
– Otoscopy
– CT
• Treatment
– Conservative
– Excision with meatoplasty
– Postaural approach

43. Osteoma
• Smooth, rounded & pedunculated
• Single
• Unilateral
• Arise from lateral part of bony EAC
• Asymptomatic
• Diagnosis
• Management

44. Other benign tumours:
• Keratoacanthoma
– Relatively common
– Arise from hair follicle
– Elevated mass, fleshy colored or pinkish
– Linked to chemical carcinogens, sun exposure, trauma & viral
– Premalignant
– Complete excision
• Papilloma: squamous papilloma and inverted papilloma
• Pleomorphic adenoma
• Choriostoma: commonly salivary gland tissue, not a true
neoplasm, no aggressive potential, surveillance

45. • Haemangioma
• Langerhans’ cell histiocytosis
• Nerve sheath neoplasm
• Paraganglioma
• Hamartoma
Haemangioma
Hamartoma

46. • Meningioma
• Chordoma
• Haemangiopericytoma
• Fibrous dysplasia:
 Fibrous tissue, bony spicules- undergoing resorption and
formation
 Islands of cartilage replacing bone marrow
 Monostotic and polyostotic
 Mc Cune Albright syndrome
 ‘Ground glass’ appearance
 Surgical excision

47. Malignant Tumours of Temporal Bone
• Squamous cell carcinoma
• Basal cell carcinoma
• Adenocarcinoma
• Acinic cell carcinoma
• Adenoid cystic carcinoma
• Melanoma
• Osteosarcoma
• Ewing’s sarcoma
• Chondrosarcoma
• Rhabdomyosarcoma
• Metastases to temporal bone
• Lymphoma
• Malignant neuroma
• Malignant paraganglioma
• CNS malignancy
• Endolymphatic sac tumour

48. Pathophysiology/Risk factors:
• Risk factors - not well defined
• Chronic otitis media:
Strongly suggests but not invariably associated
• Human papilloma virus: HPV 16 and 18 (Jin et al., 1997)
• Radium dial workers
• External beam radiation
• Ultraviolent radiation and other forms of radiation: no
strong link?

49. • Incidence: old age
EAC
• Squamous cell carcinoma: most
common
• Hidradenocarcinoma
• BCC, melanoma & mucoepidermoid
carcinoma
Middle ear
• SCC (80%), adenocarcinoma, BCC &
adenoid cystic carcinoma
Well differentiated SCC of EAC

50. Behavior of SCC of external auditory canal & middle ear
• Escape from the EAC and middle ear
cleft several directions
• Superiorly
• Posteriorly
• Anteriorly
• Inferiorly
• Medially
• Laterally

53. MRI coronal view of the temporal bone showing mass from
the left mastoid area extending out to involve external ear
canal and skin

54. MRI axial view of the temporal bone showing tumour
eroding left lateral semicircular canal

55. MRI axial view showing the left temporal lobe involvement
and cerebellar compression

56. Signs & Symptoms
• Change in pattern: may be only clue
• Growth
• Pain
• Discharge
• Deteriorating hearing
• Facial paralysis, hemi facial spasm
• Lower cranial nerve palsy
• Trismus
• Lymphadenopathy
Differential diagnosis: Otitis Externa,
COM
An exophytic mass within EAC

57. (Gustafson ML and Pensak ML, 2002)

58. Investigations
• Multimodality imaging
• CT + MRI
– Coronal: EAC, Outer Attic Wall, Tegmen
tympani
– Axial: Posterior & medial invasion of petrous
pyramid, carotid canal, lymph node detection,
prevertebral spread
• HPE: Multiple biopsy
• FNAC (CT guided)
• Angiography
– Major vessel invasion
• Audiometry: Every patient

59. Staging
(Ariaga et al., 1990)

60. (Pensak et al., 1996)

61. Treatment
• Surgical treatment followed by radiotherapy
• Extent of surgery controversial
• En block Vs Piecemeal
• Radical mastoidectomy
• Problem of local recurrence
• Extensive surgery Complete temporal bone resection

62. Sleeve Resection
• Tumours confined to skin & soft
tissue of cartilaginous portion of
EAC
• T 1
• Red line
• Incision – medial and lateral
• Involved skin & underlying cartilage
resectedwide meatoplasty
• Split-thickness skin graft

63. Lateral Temporal Bone Resection
• Tumours involving bony & cartilaginous
part (not involved annulus & tympanic
cavity)
• T 2
• Entire external auditory canal + TM +
Malleus + Incus  resected ‘en-bloc’
(Blue Line)
• Facial nerve dissected from stylomastoid
foramen to pes anserinus
• Superficial parotidectomy
• Eustachian tube - plugged

66. En-bloc lateral temporal bone resection with radical neck dissection

67. Modified Lateral Temporal Bone Resection
• Tumor extension to tympanic cavity or mastoid air cell
• T3
• Facial nerve sacrificed
• Posterior petrosectomy- bone removal posteriorly back to the
transverse sinus & posterior fossa dura
• Tegmen tympani removed
• Perilabyrinthine, retrofacial cells opened down to jugular bulb
• More extensive defect: temporalis and sternocleidomastoid
muscle flaps rotated inobliterate the defect

68. Subtotal Temporal Bone Resection
• Extensively encroach tympanic
cavity
• T3
• Entire temporal bone lateral to
petrous carotid artery en bloc
• If necessary: Portion of dura,
sigmoid sinus, parotid gland,
ramus of mandible, subtotal
parotidectomy
• Facial nerve transected

69. Total Temporal Bone Resection
• Sacrifice of carotid artery
• T 4
Light green line
• Most important decision is not to
operate at all (Gustafson et al., 2003)
• Invasion of cavernous sinus, ICA,
infratemporal fossa, paraspinous
muscle-surgically incurable

70. Radiation therapy
• Radiotherapy for curative treatment – limited success
• 5-year cure rate RT alone 28.7%, RT+ Surgery 59.6%
(Zhang et al.,1999)
• Higher doses- toxicity to brainstem
• Adjuvant therapy & palliation
• Side effects and complications- osteoradionecrosis, facial nerve
pasly & brain necrosis
• No randomized study-effect of radiation on survival & recurrence

71. Treatment of metastasis
• Regional & distant metastasis from temporal bone malignancy-low
• Nodal diseases at the time of presentation- 9% to 18%
(Pensak ML, 2003)
• Upper jugulodiagastric & parotid nodes- most commonly involved
• Neck dissection - no improvement in survival
• Superficial parotidectomy in every patient with more than
superficial disease & neck dissection reserved for known
adenopathy

72. Other epithelial malignancy:
• BCC
• Melanoma
Glandular malignant lesion
• Adenoid cystic carcinoma- most common
glandular malignancy in EAC
• Ceruminous adenocarcinoma
• Mucoepidermoid carcinoma
Sarcomas:
Fibrosarcoma, osteogenic sarcoma,
Ewing’s sarcoma, Kaposi’s sarcoma
& chondrosarcoma
Meatastasis to temporal bone: breast, lung,
kidney, stomach, brochus & prostate
Secondary tumor of temporal bone
Spreading from adjaent areas: Parotid,
nasopharyngeal, auricular & meningioma

74. Tumours in childhood
Rhabdomyosarcoma (RMS)
• Rhabdo-rod shaped, myo- muscle
• Most common soft tissue sarcoma in children
• Ear 3rd
most common after nasopharynx & orbit
• Meningeal, parameningeal & orbital
• Majority - before 12 years (average age - 4.4 years)
• Types:
1. Embryonal–nearly all of the head & neck rhabdomyosarcoma
2. Alveolar – worst prognosis
3. Pleomorphic
4. Botryoid – best survival

75. RMS contd…
Clinical features: (Prat J, 1997)
1. Mass in ear region 56%
2. Aural polyp 54%
3. Ear discharge 40%
4. Bleeding from ear 30%
5. Ear pain 22%
6. Hearing loss 14%
7. Facial paralysis 14%
Diagnosis
• Biopsy
• PCR
• Bone marrow
• CT
• MRI

76. International Rhabdomyosarcoma Study (IRS)
Based on tumour resectability:
• Group I - Tumour completely removed
• Group II - Microscopic residual tumour, involved regional nodes,
or both
• Group III - Gross residual tumour
• Group IV - Distant metastatic disease

77. Treatment
• Multimodality & multidisciplinary
• Triple therapy
• Surgery: biopsy to confirm the diagnosis
• Chemotherapy: Vincristine, Dactinomycin,
Cyclophosphamide, Ifosfamide
• Radiotherapy: Intensity Modulated Radiotherapy (IMRT)