Tumours of External Auditory Canal and Middle Ear

Tumours of External Auditory Canal and Middle Ear

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

2. 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.

3. TUMOURS OF EXTERNAL EAR

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

5. TUMOURS OF AURICLE - BENIGN Sebaceous cyst – post auricular sulcus Dermoid cyst – over upper part of mastoid Keloid – follow trauma . Treatment comprises of: Surgical excision and Injection of triamcinolone.

6. TUMOURS OF AURICLE- BENIGN 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.

7. 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.

8. 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

9. TUMOURS OF AURICLE-MALIGNANT 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

10. TUMOURS OF AURICLE-MALIGNANT 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.

11. 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.

12. EXOSTOSIS

13. TUMOURS OF EAC- BENIGN 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.

14. OSTEOMA

15. TUMOURS OF EAC- BENIGN 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.

16. TUMOURS OF EAC- BENIGN 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.

17. 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

18. 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 .

19. TUMOURS OF E.A.C- MALIGNANT 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.

20. TUMOURS OF E.A.C- MALIGNANT MALIGNANT CERUMINOMA Twice as common as benign MALIGNANT MELANOMA A very rare tumour.

21. 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.

22. 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.

23. ADENOID CYSTIC CARCINOMA

24. 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.

25. 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.

26. TUMORS OF THE MIDDLE EAR AND MASTOID 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.

27. TUMORS OF THE MIDDLE EAR AND MASTOID - 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.

28. GLOMUS TUMOUR(CONT)

29. GLOMUS TUMOUR(CONT)

30. TUMORS OF THE MIDDLE EAR AND MASTOID - BENIGN GLOMUS TUMORS (PARAGANGLIOMAS ): 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 .

31. TUMORS OF THE MIDDLE EAR AND MASTOID - BENIGN GLOMUS TUMORS (PARAGANGLIOMAS ): 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 .

32. TUMORS OF THE MIDDLE EAR AND MASTOID - BENIGN GLOMUS TUMORS (PARAGANGLIOMAS ): 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.

33. TUMORS OF THE MIDDLE EAR AND MASTOID - BENIGN GLOMUS TUMORS (PARAGANGLIOMAS ): 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.

34. TUMORS OF THE MIDDLE EAR AND MASTOID - BENIGN GLOMUS TUMORS (PARAGANGLIOMAS ): 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.

35. TUMORS OF THE MIDDLE EAR AND MASTOID - BENIGN GLOMUS TUMORS (PARAGANGLIOMAS ): 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).

36. TUMORS OF THE MIDDLE EAR AND MASTOID - BENIGN GLOMUS TUMORS (PARAGANGLIOMAS ): 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.

37. TUMORS OF THE MIDDLE EAR AND MASTOID - BENIGN GLOMUS TUMORS (PARAGANGLIOMAS ): Differential Diagnoses Because of their appearance, glomus tumors may be mistaken for: High-riding jugular bulb or dehiscent jugular bulb. Aberrant carotid artery.

38. TUMORS OF THE MIDDLE EAR AND MASTOID - BENIGN GLOMUS TUMORS (PARAGANGLIOMAS ): 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. „.

39. TUMORS OF THE MIDDLE EAR AND MASTOID - BENIGN GLOMUS TUMORS (PARAGANGLIOMAS ): 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.

40. AXIAL CT SCAN SHOWING GLOMUS TUMOUR GROWING FROM MEDIAL WALL

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

42. TUMORS OF THE MIDDLE EAR AND MASTOID - BENIGN GLOMUS TUMORS (PARAGANGLIOMAS ): 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.

43. TUMORS OF THE MIDDLE EAR AND MASTOID - BENIGN GLOMUS TUMORS (PARAGANGLIOMAS ): 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.

44. TUMORS OF THE MIDDLE EAR AND MASTOID - 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.

45. TUMORS OF THE MIDDLE EAR AND MASTOID - 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

46. TUMORS OF THE MIDDLE EAR AND MASTOID - 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.

47. TUMORS OF THE MIDDLE EAR AND MASTOID - Conditions Simulating Benign Tumors 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.

48. TUMORS OF THE MIDDLE EAR AND MASTOID - Conditions Simulating Benign Tumors 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.

49. TUMORS OF THE MIDDLE EAR AND MASTOID - 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 .

50. TUMORS OF THE MIDDLE EAR AND MASTOID - MALIGNANT Carcinoma of middle ear/EAC 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.

51. TUMORS OF THE MIDDLE EAR AND MASTOID - MALIGNANT Carcinoma of middle ear/EAC 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.

52. TUMORS OF THE MIDDLE EAR AND MASTOID - MALIGNANT Carcinoma of middle ear/EAC 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.

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

54. Squamous cell carcinoma of the middle ear and mastoid.

55. TUMORS OF THE MIDDLE EAR AND MASTOID - MALIGNANT Carcinoma of middle ear/EAC 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.

56. TUMORS OF THE MIDDLE EAR AND MASTOID - MALIGNANT Carcinoma of middle ear/EAC 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

57. TUMORS OF THE MIDDLE EAR AND MASTOID - 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.

58. Rhabdomyosarcoma of The Right Middle Ear And Mastoid

59. TUMORS OF THE 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 .

60. TUMORS OF THE MIDDLE EAR AND MASTOID - 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 .

61. TUMORS OF THE MIDDLE EAR AND MASTOID - 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.

62. THANKS