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Table of contents
Primary references, normal anatomy, normal histology
Congenital anomalies: general, accessory tragi, first branchial cleft anomalies, heterotopia
Inflammatory/infectious/autoimmune/systemic disorders: angiolymphoid hyperplasia with eosinophilia, chondrodermatitis nodularis chronica helicis, gout, idiopathic auricular ossificans, idiopathic cystic chondromalacia of auricular cartilage, inflammatory otic polyp, Kimura disease, labyrinthitis, malakoplakia, Meniere’s disease, myospherulosis, necrotizing “malignant” external otitis, otitis media, otosclerosis, Paget’s disease, pneumocystis, presbycusis, relapsing polychondritis, Wegener’s granulomatosis
External ear tumors-benign/non-neoplastic: atypical fibroxanthoma, ceruminal gland adenoma, cholesteatoma, collagenous papules, elastotic nodules, exostosis, keloid, keratinous cyst, keratoacanthoma, keratosis obturans, myxoma, osteoma, solitary fibrous tumor, synovial chondromatosis
External ear tumors-malignant: basal cell carcinoma, ceruminal gland adenocarcinoma, malignant blue nevus, melanoma, squamous cell carcinoma
Middle ear, inner ear and temporal bone tumors-benign/non-neoplastic: acoustic neuroma, cholesteatoma, jugulotympanic paraganglioma, lipochoristoma, meningioma, middle ear adenoma
Middle ear, inner ear and temporal bone tumors-malignant: adenocarcinoma, chondrosarcoma, endolymphatic sac papillary tumor, giant cell tumor, Langerhans cell histiocytosis, metastases, osteosarcoma of skull, rhabdomyosarcoma, squamous cell carcinoma
Miscellaneous: TNM staging, grossing
American Journal of Surgical Pathology (AJSP), March 1977 to December 2004
Archives of Pathology and Laboratory Medicine (Archives), January 1976 to December 2004
Human Pathology (Hum Path), March 1970 to October 2004
Modern Pathology (Mod Path), January 1988 to December 2004
Rosai, J: Ackerman’s Surgical Pathology (9th Ed); 2004
Sternberg, S: Diagnostic Surgical Pathology (4th Ed); Lippincott Williams & Wilkins, 2004
Journal search terms: ear, auditory, temporal bone
Please refer to these primary references for more detailed discussions and photographs
Sense organ for hearing and balance
Divided into external ear, middle ear and temporal bone, and inner ear
External ear conducts sound vibrations to tympanic membrane; middle ear conducts sound to auditory portion of inner ear
External ear
Consists of pinna (auricle) leading into external auditory canal, which ends at tympanic membrane
Pinna: develops from fusion of auricular hillocks, themselves from first and second branchial areas; helix is prominent rim; antihelix is prominence that is parallel with and in front of helix
External auditory canal: S shaped passage, 2.5 cm long, develops from remnant of first branchial groove; has outer cartilaginous portion and inner osseous portion
Tympanic membrane: develops from first and second branchial pouches and first branchial groove
Middle ear
Also called tympanic cavity
Filled with air (via eustachian tube); contains chain of movable bones which convey vibrations communicated to tympanic membrane across the middle ear cavity to the internal ear
Lateral aspect is tympanic membrane and squamous portion of temporal bone
Medial aspect is petrous portion of temporal bone
Superior aspect is tegmen tympani, thin plate of bone separating middle ear space from cranial cavity
Inferior aspect is thin plate of bone separating tympanic cavity from superior bulb of internal jugular vein
Anterior aspect is thin plate of bone separating tympanic cavity from carotid canal containing internal carotid artery
Posterior aspect is petrous portion of temporal bone, containing mastoid air cells and mastoid antrum
Develops from invagination of first branchial pouch (pharyngotympanic tube) from primitive pharynx
Contains auditory ossicles (malleus, incus, stapes), eustachian tube, tympanic cavity, epitympanic recess, mastoid cavity, chorda tympani of facial nerve (cranial nerve VII)
Malleus and incus develop from mesoderm of first branchial arch (Meckel cartilage), stapes develops from mesoderm of second branchial arch (Reichert cartilage)
Connects to pharynx through Eustachian tube
Connects with mastoid cavity through contiguous pneumatic spaces
Inner ear
Located in medial (petrous) portion of temporal bone
Contains cochlea (sense organ for hearing) and vestibular labyrinth (sense organ for balance with membranous and osseous portions), internal auditory canal (contains vestibulocochlear nerve, CN VIII)
Vestibular labyrinth includes blind endolymphatic sac, located in petrous bone
Endolymphatic sac is connected to utricle and saccule by endolymphatic duct, which passes along petrous bone
Develops before middle and external ear, at end of first month of gestation
External ear
Pinna: resembles skin elsewhere, with keratinized, stratified squamous epithelium, dermal adnexal structures, subcutaneous fibroconnective tissue, fat and elastic fibrocartilage which provides support
External auditory canal: lined by thin keratinized stratified squamous epithelium covering scant fibrous stroma along entire canal and covering external tympanic membrane
Outer third contains ceruminous glands (modified apocrine glands) deep within dermis that produce cerumen; glands are in clusters of cuboidal cells with intensely eosinophilic cytoplasm with apical snouts and containing golden-yellow, granular pigment and secretory droplets along luminal border; glands are surrounded by myoepithelial cells; outer canal contains cartilage not bone
Inner two thirds has very thin epidermis which lacks rete pegs, no/reduced number of ceruminous glands and dermal adnexa; contains bone not cartilage
Cerumen: watery fluid devoid of lipids; drains from glands into ducts, which open into hair sacs of ear canal hairs; fluid mixes with sebaceous gland secretions to produce cerumen (wax)
Tympanic cavity: thin fibrous structure lined by attenuated keratinizing squamous epithelium on external canal side
Middle ear
Eustachian tube: respiratory epithelium that becomes pseudostratified as it approaches nasopharynx; lymphoid component, prominent in children, is called Gerlach tubal tonsil; no glands
Tympanic membrane: thin fibrous structure lined by flat, single layer of cuboidal epithelium on middle ear side
Mastoid: flat, single, cuboidal epithelium
Ossicles: typical synovial joints
Inner ear
Vestibular labyrinth is lined by flat to low columnar epithelium overlying vascular stroma
Congenital anomalies
Common
Either isolated or with other abnormalities
Cosmetic or functional
Also called accessory or supernumerary ear, accessory auricle, polyotia
Appears at birth; may be related to second branchial arch anomalies
Associated in some cases with cleft lip/palate, mandibular hypoplasia, oculoauriculovertebral dysplasia (Goldenhar syndrome)
Solitary or multiple, unilateral or bilateral, sessile or pedunculated, soft or cartilaginous
Gross: skin covered nodule, often anterior to auricle
Micro: skin with cutaneous adnexae and central cartilage (resembles normal external auricle)
DD: squamous papillomas (no cartilage, no adnexae)
First branchial cleft anomalies
Cysts, sinuses and fistulas near external ear; may be pre-, post- or infraauricular; also at angle of jaw, at ear lobe, in external auditory canal or parotid gland
Fistulas may connect skin with external auditory canal
1-8% of branchial defects
Usually middle aged women
Type I: keratinizing squamous epithelium without adnexae (ectoderm only, duplicates membranous external auditory canal)
Type II: keratinized squamous epithelium with adnexa and cartilage (ectoderm and mesoderm, duplicates external auditory canal and pinna)
Treatment: excision; often recurs
Normal appearing tissue in abnormal anatomic location
Middle ear heterotopias include salivary gland tissue and neuroglial tissue
Salivary gland heterotopias usually occur in women, associated with facial nerve and ossicle anomalies, suggesting a second branchial arch developmental anomaly
Neuroglial heterotopia often represents an acquired encephalocele with herniation of brain into middle ear and mastoid; treatment is surgical, although tissue may adhere to facial nerve
Case reports: salivary gland heterotopia of middle ear (Archives 1982;106:39)
Inflammatory/infectious/autoimmune/systemic disorders
Angiolymphoid hyperplasia with eosinophilia
Also called epithelioid (histiocytoid) hemangioma
Benign angiomatous subcutaneous process, usually of auricle and external canal; also elsewhere in head and neck
Usually men and women in 20’s to 40’s, may have history of trauma
Symptoms: pruritis and bleeding after scratching
Treatment: local excision or desiccation are curative
Gross: pink-red-brown cutaneous papules or subcutaneous nodules up to 1 cm; may coalesce to form plaque-like lesions
Micro: unencapsulated but circumscribed; dermal, nodular proliferation of granulation type tissue with haphazard, small caliber, irregularly shaped blood vessels with epithelioid endothelial cells containing hyperchromatic nuclei; also patchy lymphocytes, eosinophils and histiocytes
DD: hemangioma (no epithelioid endothelial cells, no inflammatory infiltrate), angiosarcoma (anastomosing vascular channels lined by pleomorphic cells with increased mitotic activity, no inflammatory infiltrate)
Chondrodermatitis nodularis chronica helicis
Also called Winkler disease
Idiopathic, nonneoplastic ulcerative lesion of auricle
Usually men ages 40+; uncommon in women
Symptoms: appear spontaneously; unilateral, painful nodule
Treatment: wedge or cartilage excision; glucocorticoid injection
Gross: dome shaped nodule, 0.3 to 1.8 cm, with crusty scale covering central area of ulceration
Micro: central ulceration of epidermis with adjacent acanthosis, hyperkeratosis, parakeratosis and pseudoepitheliomatous hyperplasia; base of ulcer has granulation tissue that usually involves perichondrium and cartilage; no dermal adnexa at site of lesion; may have foci of fibrinoid necrosis; vascular proliferation may resemble a glomus tumor
DD: clinically resembles carcinoma
Primary gout (90%): idiopathic (85%) with overproduction of uric acid (may have normal excretion) or known enzyme defects (5%, partial hypoxanthine-guanine phosphoribosyl transferase deficiency [HGPRT])
Secondary gout (10%): increased nucleic acid turnover due to leukemia/lymphoma, chronic renal disease, HGPRT deficiency
Xray: no calcifications
Laboratory: elevated urinary uric acid, leukocytosis, increased erythrocyte sedimentation rate
Gouty tophi (depositions of sodium urate) commonly deposit in helix of ear as painful, skin-covered, firm nodule
Micro: tophi are composed of needle-shaped aggregates of urate crystals with surrounding foreign body giant cell reaction; urate crystals dissolve with routine processing, so fix a smear of crystals in absolute alcohol or nonaqueous fixation; no birefringence
DD: pseudogout (rhomboid or needle shaped, weak positive birefringence with polarized light, radiographic calcifications)
Idiopathic auricular ossificans
Ectopic calcification of auricle (“rigid ear”) of unknown etiology
Very rare (<20 cases reported)
Case reports: 60 year old man with 10 year history of slowly stiffening auricles (Archives 2004;128:1432)
Micro: cartilaginous replacement by bone
DD: specific causes (frostbite, physical trauma, inflammatory conditions, Addison’s disease)
Idiopathic cystic chondromalacia of auricular cartilage
Also called auricular or endochondral pseudocyst
Benign cystic degeneration of auricular cartilage of unknown cause
Usually men age 20-40 years
Unilateral swelling of cartilage over weeks to years, most commonly on scaphoid fossa of auricle
May be due to minor trauma
Treatment: excision
Micro: fluid filed distended mass composed of cyst-like wall with fibrous and granulation tissue lining but no epithelium; cyst contains 1-2 mm rim of cartilage; cyst fluid resembles olive oil; no/mild atypia
DD: relapsing polychondritis, subperichondrial hematoma, chondrodermatitis nodularis helicis chronicus
References: Archives 1986;110:740
Inflammatory otic or aural polyp
Inflammatory polypoid proliferation of middle ear mucosa secondary to chronic otitis media
May perforate tympanic membrane and appear to originate from external auditory canal; with time, may destroy ossicles
More common in children, but occurs in all ages
Treatment: excision
Gross: polypoid, soft/rubbery, pink/tan/red lesion
Micro: squamous or ciliated columnar epithelium containing lymphocytes, histiocytes, plasma cells, eosinophils; also Mott cells (plasma cells with large eosinophilic immunoglobulin) and granulation type tissue; variable neutrophils, multinucleated giant cells, cholesterol granulomas and tympanosclerosis
DD: plasmacytoma (monoclonal light chains)
Asians, usually males
Large subcutaneous nodules, usually not in head and neck, with regional lymphadenopathy and peripheral eosinophilia
Micro: prominent lymphoid proliferation, sparse vascular proliferation; often extends to fascia and skeletal muscle; adipose tissue is often fibrotic; eosinophils are common
DD: angiolymphoid hyperplasia with eosinophils (lesion more superficial, may have rare/no eosinophils), angiosarcoma
Inflammation of inner ear secondary to various causes
Serous: mildest form; due to local irritant, such as acute or chronic otitis media without bacterial invasion of inner ear, temporal bone or meningitis; has accumulation of granular eosinophilic material within labyrinth or perilymphatic spaces, with mild endolymphatic hydrops
Suppurative: neutrophils and bacteria are present in perilymphatic spaces; may destroy sensory end organs and membranous labyrinth
Chronic: due to local osteitis of otic capsule secondary to prior acute suppurative labyrinthitis or chronic inflammation of membranous labyrinth
Ossifying: end stage of suppurative labyrinthitis, with ossification of labyrinthian structures but no inflammatory infiltrate
Viral: due to mumps, measles, CMV; viral cytopathic changes are present in scala media
Complications include involvement of intracranial structures (meningitis, venous thrombophlebitis, intracranial abscess, facial nerve paralysis, otic hydrocephalus)
Case reports: CMV endolabyrinthitis in premature male newborn (Archives 1977;101:118)
Rarely occurs in middle ear
Due to inability of histiocytes to ingest bacteria such as E coli, with accumulation in phagolysosomes
Micro: sheets of histiocytes with eosinophilic, granular to vacuolated cytoplasm (Hansemann cells), lymphocytes, plasma cells, neutrophils; diagnostic feature is intracytoplasmic or extracellular calcospherites (Michaelis-Gutmann bodies)
Positive stains: Michaelis-Gutmann bodies are PAS+ diastase resistant, Prussian blue/iron+, von Kassa/calcium+
Also called endolymphatic hydrops, idiopathic endolymphatic hydrops or Lermoyez syndrome
Idiopathic disorder of inner ear associated with spontaneous attacks of vertigo, sensorineural hearing loss, tinnitus and sensation of aural fullness
Incidence varies from 7.5 per 100K in France to 157 per 100K in England; 60% women, peaks in 40’s to 60’s, but wide age range
Associated with HLA B8/DR3
May be due to accumulation of endolymph in membranous labyrinth, perhaps due to inadequate absorption by endolymphatic sac
Treatment: dietary modification, intermittent dehydration, diuretics, vasodilators in increase microcirculation of ear, reduction of immunoreactivity; 60-80% improve; surgery includes shunting and decompression of endolymphatic sac, labyrinthectomy, sectioning of vestibular nerve
Micro: initially involves cochlear duct and saccule; later entire endolymphatic system with dilation, rupture and collapse of membranous labyrinth with possible fistula; may have severe atrophic changes with loss of cochlear neurons
Iatrogenic due to petrolatum based ointments
Usually nasal cavity and paranasal sinuses; occasionally affects middle ear
History of recent surgery with packing of area
Treatment: symptomatic
Micro: pseudocysts within fibrous tissue with lymphocytes, histiocytes, giant cells and plasma cells; pseudocysts contain “parent bodies” containing numerous spherules
Negative stains: GMS for fungi
Necrotizing “malignant” external otitis
Potentially fatal external otitis due to Pseudomonas aeruginosa, Aspergillus or other fungal infection
Usually older patients, often with diabetes, chronic debilitation or immunodeficiency
Initially affects external auditory canal with symptoms of acute otitis externa; later pain, purulent otorrhea and swelling; may progress to cellulitis, chondritis, osteomyelitis, involve middle ear space or base of skull, and cause cranial nerve palsies, meningitis, venous thrombosis or brain abscess
Up to 75% mortality if treatment is delayed
Due to tissue ischemia (from primary pathologic state above) plus neutrophilic migratory defect plus virulence of Pseudomonas
Treatment: antibiotics, surgical debridement
Case reports: oxalate crystals within necrotic tissue are associated with Aspergillus niger infection (Mod Path 1993;6:493)
Gross: ulcerated skin near osseous portion of external auditory canal, often with abundant necrotic and granulation tissue
Micro: epithelium is necrotic or ulcerated with pseudoepitheliomatous hyperplasia, marked mixed inflammatory infiltrate in subcutaneous tissue, necrotizing vasculitis; also necrotic bone and cartilage with heavy inflammatory infiltrate in viable bone; variable sequestra of nonviable bone or cartilage
Positive stains: gram stain (gram negative rods)
DD: squamous cell carcinoma
Acute or chronic infectious disease of middle ear
Usually childhood disease caused by Streptococcus pneumoniae or Haemophilus influenza
Hyperemic, opaque and bulging tympanic membrane with limited mobility; may have purulent otorrhea
Infection probably occurs post-pharyngitis via eustachian tube
Severe cases are associated with destruction of ossicles
Tympanosclerosis: dystrophic calcification of tympanic membrane or middle ear associated with recurrent cases of otitis media, occurs in 3-33% of cases; may be reversible in children, usually irreversible in adults and associated with conductive hearing loss
Rarely caused by other organisms such as fungi, Pneumocystis in HIV+ patients or viruses
Treatment: antibiotics; complications of mastoiditis, labyrinthitis, meningitis or abscess are now rare
Gross: not a common specimen, but may have small fragments of soft/rubbery granulation tissue
Micro: acute and chronic inflammatory cells, haphazard glandular metaplasia with cilia, fibrosis, hemorrhage, foci of calcification (tympanosclerosis), cholesterol granulomas and reactive bone formation
Cholesterol granulomas: foreign body granulomas in response to cholesterol crystals from rupture of red blood cells and breakdown of lipid bilayer in cell membrane, prominent cholesterol clefts; associated with interference to drainage or ventilation of middle ear space; not related to cholesteatomas
DD: middle ear adenoma (regular, not haphazard glands, no cilia)
Disorder of bone remodeling affecting bony labyrinth and stapes footplate
Causes fixation of stapes footplate in oval window and inability to transmit sound waves, manifesting as conductive hearing loss
Usually women; 50% have family history; begins in teenagers and slowly progresses
More common in whites than blacks, Asians or Native Americans
85% bilateral
Treatment: stapedectomy (correction of fixation of footplate of stapes)
Gross: specimens are usually head and crura of stapes, which are not affected by disease
Micro: initially bone resorption and replacement by cellular fibrovascular tissue around blood vessels; then immature bone is deposited with continuous resorption and remodeling; over time, bone is deposited with increased collagen and reduced ground substance, resulting in densely sclerotic bone with prominent cement lines
Also called osteitis deformans
Chronic progressive disease of unknown cause, affects skull and temporal bone in 70% of cases
Causes enlargement and tortuosity of superficial temporal artery and its anterior branches
Also affects numerous structures of external, middle and inner ear
Affects 3% of population ages 40+, 11% at age 80+; men affected slightly more than women
1% have transformation to osteosarcoma or other sarcoma, with 5 year survival of < 10%
Micro: osteolytic phase has extensive osteoclastic activity and bone resorption; mixed/combined phase has osteoblastic activity exceeding osteoclastic activity; osteoblastic phase has dense new bone with mosaic pattern of cement lines
DD: otosclerosis (younger age, doesn’t affect skull)
Case report in HIV+ patient with infection of middle ear (Archives 1992;116:500)
Hearing loss, often due to cochlear hair cell degeneration
Usually no surgical specimen
Also called polychondropathia
Uncommon systemic episodic or relapsing disease with progressive degeneration of cartilage throughout the body
Probable autoimmune process (? antibodies to type II collagen) associated with other autoimmune disorders
Whites, no gender preference, usually symptomatic in 40’s to 60’s, although affects all ages
90% have involvement of auricular cartilage, usually bilateral, with swelling, erythema and tenderness
Variable relapsing of disease
May cause cauliflower ear and saddle node deformities
Clinical diagnosis requires 3 of the following - (a) recurrent chondritis of both auricles; (b) nonerosive inflammatory arthritis; (c) chondritis of nasal cartilage; (d) ocular inflammation including conjunctivitis, keratitis, scleritis, episcleritis or uveitis; (e) chondritis of upper respiratory tract including larynx or tracheal cartilage; (f) cochlear or vestibular damage with sensorineural hearing loss, tinnitus or vertigo
Laboratory: nonspecific elevated sedimentation rate, mild leukocytosis, normochromic normocytic anemia; variable elevated ANCA
Prognosis varies from prolonged course to aggressive and fulminant disease leading to death from respiratory tract or cardiovascular involvement (aortic insufficiency)
Treatment: responds to steroids or dapsone (this also confirms diagnosis); advanced cases require immunosuppressive agents
Micro: mixed inflammatory infiltrate (lymphocytes, plasma cells, neutrophils, occasional eosinophils) extending into cartilage with blurring of interface between cartilage and adjacent soft tissue; cartilage shows loss of normal basophilia, loss of chondrocytes and destruction of lacunar architecture at advancing edge of inflammation, with cartilage replaced by fibrous tissue
Positive stains: granular deposition of IgG and C3 in perichondrial fibrous tissue (Hum Path 1980;11:19)
Systemic necrotizing vasculitis that typically involves kidneys, lung, upper aerodigestive tract
Otologic involvement (otitis media, tympanic membrane perforation, sensorineural hearing loss, perforation of ear lobes, external otitis), as well as facial palsy occurs in 20-60% who have disease at traditional sites
Laboratory: elevated serum ANCA
Treatment: corticosteroids, immunosuppressive drugs may cause long term remission and reverse hearing loss and facial palsy
Micro: vasculitis, necrosis or granulomatous inflammation (few cases have all 3)
DD: polyarteritis nodosa (necrotizing vasculitis of small to medium sized muscular arteries), rheumatoid arthritis (conductive hearing loss due to involvement of incus-maleus and incus-stapes articulations)
External ear tumors-benign/non-neoplastic
Also called superficial malignant fibrous histiocytoma
Usually sun damaged skin of elderly (75% in head and neck) or superficial soft tissue of extremities in young
Treatment: complete excision has excellent prognosis; local recurrence if incomplete excision; probably cannot metastasize; may recur as large mass in deep soft tissue, which should be treated as malignant fibrous histiocytoma (not superficial)
Gross: asymptomatic firm nodule 1-2 cm, often with ulceration
Micro: nonencapsulated but circumscribed spindle cell proliferation of dermis; cells are spindled or pleomorphic with bizarre multinucleated forms or hyperchromasia; cells may have foamy cytoplasm; increased mitotic figures, including atypical forms; may have vascular invasion; no junctional activity, no necrosis
Negative stains: keratin, S100, HMB45, desmin; variable actin
DD: spindle cell carcinoma, spindle cell melanoma, leiomyosarcoma (desmin+), malignant fibrous histiocytoma (> 2 cm, extensively infiltrative and either necrosis or vascular invasion)
Arise from cerumen-secreting modified apocrine glands of external auditory canal
Uncommon in general, but most common external auditory canal tumor in outer portion where ceruminal glands exist
Slightly more common in men, mean age 52-54 years, range 12-85 years
Location of tumor (parotid gland, middle ear, external auditory canal) is important because treatment differs
Symptoms: slow growing external auditory canal mass or blockage with conductive hearing loss
Treatment: complete surgical excision; recurrences are due to incomplete excision
Gross: skin covered, circumscribed, polypoid or rounded masses, gray-white-pink, 0.4 to 2 cm; usually not ulcerated; specimens are usually received by pathologist in small fragments without obvious surface epithelium
Micro: unencapsulated but well circumscribed glandular proliferations in cribriform, solid, cystic or papillary patterns; glands composed of inner cuboidal or columnar cells with eosinophilic cytoplasm and apical snouts (decapitation type secretion) and outer spindled myoepithelial cells with hyperchromatic nuclei; inner cells contain yellow-brown granular cerumen pigment; hyalinized stroma present; no prominent pleomorphism or mitotic figures, no invasion or necrosis
Positive stains: cerumen is PAS+ or mucicarmine+; luminal cells are CK7+, CD117/kit+; basal cells are p63+, CK5/6+, S100+
DD: ceruminal adenocarcinoma (more infiltrative; perineural invasion, irregular gland formation, pleomorphism with prominent nucleoli, increased mitotic figures including atypical mitotic figures, tumor necrosis; usually no ceruminous granules), middle ear adenoma, parotid gland tumor, paraganglioma (nested pattern of paraganglia cells supported by sustentacular cells; chromogranin+, S100+)
References: AJSP 2004;28:308
Ceruminal gland pleomorphic adenoma
Uncommon
Micro: resembles salivary gland tumor with mixture of epithelial and myoepithelial cells in myxoid stroma
Syringocystadenoma papilliferum
Benign tumor of apocrine gland origin
Usually scalp or face, but also external auditory canal
Micro: similar to tumor at other sites - papillary architecture with marked plasma cell infiltrate
Rare; differs from middle ear cholesteatoma
Cystic mass of keratinized squamous epithelium overlying area of bone sequestration in external auditory canal
DD: keratosis obturans
Bilateral, smooth, firm, small papules on inner aspects of pinna
Rarely involves external auditory canal
Micro: dense collagen with dilated vessels and scattered fibroblasts
Small papules and nodules, often on antihelix
Due to actinic damage
Micro: dermal clumps of elastic tissue
Reactive, localized overgrowth of bone, with a broad base
Called osteoma if pedunculated
Arise from wall of external auditory canal
Usually multiple and bilateral; asymptomatic until large enough to block external auditory canal
Highest incidence in cold water swimmers and surfers in Australia and New Zealand
Treatment: medical (for external otitis), transmeatal surgical excision if medical treatment fails
Gross: broad based, mound-like bony proliferation resembling normal cortical bone (however pathologist usually only gets fragments); no bone marrow spaces; bone is covered by periosteum with overlying thin skin
DD: osteoma (uncommon in ear)
Greek for “crab claw”
Exaggerated, non-neoplastic scarring response to trauma
Common in young black women after ear piercing
Treatment: excision (although 40% recur), intralesional steroids or interferon
Gross: polypoid, covered by thin, hairless skin; usually < 2 cm
Micro: nonencapsulated; haphazard fascicles of dense, hyalinized collagen that appear edematous due to dermal mucosubstances; scattered fibroblasts and myofibroblasts; blends with adjacent dermal tissue; overlying epithelium is thin and lacks adnexae; widely scattered dilated blood vessels
DD: hypertrophic scar (delicate fibrillar collagen in orderly arrangement, usually no abundant mucosubstances; don’t recur), dermatofibroma (more cellular), dermatofibrosarcoma protuberans (more cellular, usually pseudoepitheliomatous hyperplasia of overlying epidermis)
Common around external ear
Related to branchial cleft or epidermal inclusion cysts
Micro: lined by keratinized squamous epithelium, filled with keratin
Common in skin of external ear
Accumulation of keratin debris deep within external auditory canal, which may cause bone remodeling and inflamed epithelium
Older individuals than cholesteatomas
Treatment: removal of keratin plug
Micro: tightly packed keratin squames in lamellar pattern; diffuse acanthosis and hyperkeratosis of skin of canal with underlying chronic inflammatory infiltrate
May arise as part of Carney complex
Carney complex: autosomal dominant disorder with multiple cardiac and skin myxomas, spotty pigmentation of skin, endocrine overactivity (pigmented nodular adrenocortical disease, large cell calcifying Sertoli cell tumor of the testis, pituitary adenoma), blue nevi, psammomatous melanotic schwannoma, bone tumors (AJSP 1994;18:274)
Treatment: excision; usually don’t recur
Gross: mucoid, 3 mm to 2 cm
Micro: circumscribed but nonencapsulated hypocellular tumor with minimal blood vessels, composed of mucoid material with suspension of loose framework of reticulin fibers; cells are spindled with tiny pyknotic nuclei and delicate cytoplasmic processes; no pleomorphism; may have pseudocapsule of condensed reticulin fibers and compressed skeletal muscle
Positive stains: Alcian blue (myxoid matrix), mucicarmine, colloidal iron, vimentin
Negative stains: skeletal muscle, S100
DD: myxoid change in neurofibroma, schwannoma or lipoma; sarcomas (more cellular, more vascular, atypia, mitotic figures)
True neoplasms capable of unlimited growth, in contrast to exostosis
Asymptomatic solitary masses attached by narrow pedicle to tympanosquamous or tympanomastoid suture line
Micro: mature bone with bone marrow and intraosseous fibrovascular tissue covered by keratinized squamous epithelium
Slow growing tumor, usually benign, although malignant transformation and metastases have been reported at other sites
Only one case report in auditory canal
Case reports: 39 year old woman with tumor of auditory canal (Archives 2004;128:e169)
Micro: fascicular, whorled or haphazard (patternless) arrangement of oval/spindle cells in myxoid or collagenous stroma with inflammatory cells and vascular clefts; may have mild atypia or occasional mitotic figures
Positive stains: vimentin, CD34, bcl2, CD99
Negative stains: keratin, EMA, S100, CD31, neurofilament
Also called synovial chondrometaplasia, synovial osteochondromatosis
Possibly neoplastic process in which multiple cartilaginous nodules form in synovium, some detach and float in joint space
Temporomandibular joint (TMJ) lesions cause asymptomatic mass of external auditory canal, with preauricular swelling and limited joint motion; usually in adult women
Xray: numerous radiopaque loose bodies within TMJ, but without bone destruction
Confined to joint space, usually easily enucleated; rarely extends into parotid gland, temporal bone, cranium, auditory canal
Treatment: conservative surgery
Gross: synovium with diffuse polypoid or pedunculated nodules, 1 mm to 3 cm, with smooth to granular external surface
Micro: nodules of mature cartilage of varying cellularity within synovium and joint space; cartilage may have atypia, hyperchromasia, binucleated chondrocytes, mitotic figures; also calcification and ossification
DD: chondrosarcoma
External ear tumors-malignant
Common tumor of auricle and external auditory canal
Untreated tumors may extend into middle ear, mastoid or cranial cavity
Treatment: surgery, radiation therapy
Ceruminal gland adenocarcinoma
Usually men, ages 30-59 but wide age range
Associated with local pain
Tend to recurrence locally; only rarely metastasizes to regional lymph nodes and lung
Adenoid cystic carcinoma subtypes usually have relatively good 5 year survival, but poor 10- and 20-year survival
Treatment: en bloc resection; more radical surgery if middle ear or temporal bone involvement; also radiation therapy
Case reports: 38 year old man with ceruminous adenoid cystic carcinoma and a contralateral brain metastasis (Archives 2002;126:87)
Micro: loss of glandular double cell layer, as only luminal epithelial cells are present; pleomorphism, nuclear anaplasia, mitotic activity and invasive growth are evident except in well differentiated tumors, which may resemble adenomas except for invasive growth; variants include adenoid cystic carcinoma and mucoepidermoid carcinoma
DD: direct extension of parotid gland tumors; dermal eccrine cylindroma, paraganglioma of middle ear
Derived from benign cellular blue nevus, a dermal melanocytic proliferation thought due to arrested migration of immature dermal melanocytes during embryogenesis
Case reports: 11 year old with malignant blue nevus of left ear associated with large multinodular blue nevus at same locus and 2 intracranial melanocytic tumors (Hum Path 2004;35:1292)
Usually auricle
Usually superficial spreading type
Squamous cell carcinoma of external auditory canal
15% of primary cutaneous carcinomas of external ear and auditory canal
Tumors of external ear are more common in men; tumors of canal are more common in women
Usually age 60+ years
Poor prognostic factors: > 2 cm, depth > 4 mm, poorly differentiated tumors, perineural invasion, development within a scar, previously treated squamous cell carcinoma at the site, immunosuppression. location within inner portion of canal with deep involvement of temporal bone
Tumor spread: tumors of helix spread along helix, to antihelix, to posterior surface of ear; tumors of antihelix spread concentrically; tumors of posterior surface spread to helix; tumors of canal tend to invade bone, may destroy tympanic membrane and penetrate middle ear
Treatment: complete excision (mastoidectomy or temporal bone resection for canal tumors), possibly radiation therapy
Often recurs (19%) or metastasizes (11%); death may occur due to intracranial extension
Gross: polypoid, firm/rubbery nodules, frequent ulceration
Micro:
Well differentiated: most common, composed of infiltrating nests of cells with keratin pearls or individual cell keratinization and intercellular bridges; variable nuclear atypia; frequent mitotic activity with atypical forms; invasion may be superficial with irregular budding of basal epithelium or irregular tongues of tumor projecting downward
Moderated differentiated: scattered individually keratinized cells but no keratin pearls
Poorly differentiated: no obvious keratinization, but squamous epithelial dysplasia, pavement-like cellular pattern, foci with intercellular bridges
DD: irritated seborrheic keratosis, melanoma, malignant fibrous histiocytoma
Spindle cell carcinoma
Also called sarcomatoid carcinoma
Micro: infiltrating tumor with interlacing bundles or fascicular growth; spindled and epithelioid cells with amphophilic or eosinophilic cytoplasm, pleomorphic and hyperchromatic nuclei, increased N/C ratios, frequent mitotic activity with atypical forms; often surface ulceration, surface epithelial dysplasia and differentiated squamous cell carcinoma; may produce chondroid or osteoid matrix
Positive stains: keratin, EMA, vimentin
Negative stains: S100, HMB45
Adenoid squamous cell carcinoma
Unusual variant
Often face and scalp, particularly periauricular area
Micro: pseudoglandular appearance due to tumor cell acantholysis in center of tumor nests; usually dysplastic surface epithelium
Negative stains: mucin
Middle ear, inner ear and temporal bone tumors-benign/non-neoplastic
Also called schwannoma, neurilemmoma, benign peripheral nerve sheath tumor
Benign neoplasm originating from Schwann cells of cranial nerve VIII, superior or vestibular branch
10% of intracranial neoplasms, 90% of tumors at cerebellopontine angle
Usually women, more common in age 30’s to 60’s, but wide age range
Symptoms: sensorineural hearing loss, tinnitus, loss of equilibrium; may eventually compress adjacent cranial nerves (V, VII, IX, X, XI), cerebellum, brainstem
8% are bilateral (associated with neurofibromatosis type 2)
16% have symptoms of neurofibromatosis; these patients develop acoustic neuromas in teens
Xray: flaring, widening or erosion of internal auditory canal
Treatment: complete excision
Rarely malignant (associated with neurofibromatosis)
Gross: circumscribed, tan-white-yellow, rubbery/firm, up to 5 cm, variable cystic change
Micro: unencapsulated but otherwise resemble schwannomas at other locations; interlacing fascicles of cells with indistinct cytoplasmic borders, elongated and twisted nuclei; Verocay bodies (whorling or palisading of nuclei); hyalinization of vessels; commonly have regressive changes (cellular pleomorphism with hyperchromasia, cystic degeneration, necrosis, calcification, hemorrhage); may be highly cellular (cellular schwannoma); no/rare mitotic figures
Positive stains: S100 (diffuse, strong)
Negative stains: keratin, chromogranin, synaptophysin
Also called keratoma (more accurate term), epidermal inclusion cyst of middle ear
Stratified squamous epithelium that forms saclike accumulation of keratin within middle ear space
May cause progressive bone erosion of ossicles and surrounding bone, but not neoplastic; incomplete excision may cause widespread bone destruction
Damage apparently due to associated inflammation and proteolytic enzymes, not pressure from tumor mass (Mod Path 2001;14:1226)
Usually men, ages 20’s to 30’s
May be congenital, post-middle ear infection or unknown cause
Treatment: complete excision of all components
Gross: cystic, white masses of varying size with creamy or waxy granular material
Micro: keratinized stratified squamous epithelium with granulation tissue and keratin debris (presence of keratinizing squamous epithelium is required for diagnosis); also chronic inflammatory infiltrate, cholesterol clefts, foreign body giant cell granulomas; no dysplasia
DD: squamous cell carcinoma (atypia, desmoplasia)
Also called glomus jugulare tumor or glomus tympanicum tumor
Most common tumor of middle ear
Benign neoplasm arising from paraganglioma of middle ear or temporal bone region
Usually women, ages 40-69 years
85% arise in jugular bulb, causing mass in middle ear or external auditory canal; 12% arise from tympanic branch of glossopharyngeal nerve (Jacobson nerve), causing middle ear mass; 3% arise from posterior auricular branch of vagus nerve (Arnold nerve), causing external auditory canal mass
Usually cause conductive hearing loss
May be locally invasive into temporal bone and mastoid; may cause cranial nerve palsies, cerebellar dysfunction, dysphagia, hoarseness
Tumors are fed by branches of nearby large arteries; may bleed profusely at biopsy
Histology of benign appearing tumors does not predict behavior
15% extend intracranially
Rarely are malignant histologically (necrosis, mitotic activity, vascular invasion), with metastases to cervical lymph nodes, lung, liver
Treatment: complete excision (may be difficult) with possible preoperative embolization or radiation therapy (reduces vascularity, promotes fibrosis); 50% recur locally
Case reports: tumor with regional metastases and spinal metastases 10-13 years after presentation (Archives 1990;114:976)
Gross: polypoid, red, friable
Micro: classic organoid or nesting pattern of paragangliomas with central round/oval chief cells containing abundant eosinophilic granular or vacuolated cytoplasm, uniform nuclei with dispersed chromatin; also sustentacular cells (spindled, basophilic, difficult to see with H&E) are present at periphery of nests; prominent fibrovascular stroma separates nests; may have pleomorphism, but this does not predict malignant behavior; occasional dense fibrous stroma or apparent infiltrative growth; rare mitotic figures or necrosis; no glandular or alveolar differentiation
Positive stains: chromogranin and synaptophysin (chief cells), S100 (sustentacular cells); reticulin (stains stroma and delineates nesting pattern, particularly helpful with crushed specimens), variable vimentin (both cell types)
Negative stains: keratin, EMA, HMB45, desmin and other myogenic markers, PAS, mucicarmine
EM: neurosecretory granules
DD: other neuroendocrine tumors, melanoma, carcinoma, middle ear adenoma, acoustic neuroma, meningioma
Also called lipomatous choristoma
Rare tumor of cranial nerve VIII within acoustic canal or cerebellopontine angle
May be a congenital malformation
Indolent behavior, in contrast to acoustic neuromas or meningiomas
70% men; hearing loss and unilateral tumors
Treatment: conservative surgery with nerve preservation
Case reports: 2 cases of lipoma of internal auditory canal (probably not lipochoristoma, Archives 1996;120:681)
Gross: arise from CN VIII (cochlear or vestibular branch), pink-ivory-gray-maroon, soft-rubbery
Micro: normal nerve components (myelinated nerve branches, glia cells, neurons, small thin walled vessels), mature adipose tissue with variable mature fibrous tissue, tortuous thick walled vessels, smooth muscle bundles and skeletal muscle fibers
References: Archives 2003;127:1475
Benign tumor arising from arachnoid cells
Must exclude secondary extension from intracranial tumor
13-18% of intracranial tumors; second most common tumor of cerebellopontine angle (after acoustic neuroma)
Usually women, age 40+ years; rarely children
Ectopic meningiomas also occur, either with (secondary) or without (primary) an identifiable CNS connection
Middle ear and temporal bone are most common sites of head and neck ectopic meningiomas
Neurofibromatosis patients have increased incidence, also more likely to have multiple tumors and extracranial tumors
Excellent prognosis with overall raw survival of 15.5 years; no metastases but may recur locally
Xray: speckled calcifications in soft tissue mass
Treatment: complete excision; recur if inadequate excision
Case reports: cutaneous meningioma of external auditory canal in 48 year old woman with intracranial meningioma at ipsilateral cerebellopontine angle but no identifiable connection between the tumors (Archives 1998;122:97)
Gross: 0.5 to 4.5 cm, gray-white-pink, firm, usually fragmented into small pieces
Micro: resemble intracranial tumors; whorls, nests or lobular growth of round/oval or spindled cells with pale cytoplasm, indistinct cell borders, punched out or empty nuclei due to intranuclear cytoplasmic inclusions; psammoma bodies; often microscopic bone invasion; cholesteatoma is often present
Positive stains: EMA, vimentin
Negative stains: keratin, neuroendocrine markers
DD: paraganglioma, acoustic neuroma, carcinoma, melanoma, middle ear adenoma
References: Mod Path 2003;16:236
Rare, benign glandular neoplasm originating from middle ear mucosa
No gender preference, usually 20’s to 40’s, but wide age range
Affects all sites in middle ear
Usually no pain, discharge or facial nerve paralysis
Occasionally perforates tympanic membrane and extends into external auditory canal
Not associated with chronic otitis media or cholesteatomas
Rarely are locally aggressive, invade vital structures and cause death
Rosai believes they form a continuum with carcinoid tumor and could be considered adenocarcinoid tumors
Excellent prognosis
Treatment: complete surgical excision; mastoidectomy may be necessary for large lesions; recurs with inadequate excision
Gross: gray-white to red-brown, firm/rubbery masses; no hemorrhage, mean 0.8 cm
Micro: unencapsulated but relatively well circumscribed with variable patterns (sheets, solid, trabecular, cystic, cribriform, NOT papillary) of glands or tubules composed of single layer of cuboidal or columnar cells with variable eosinophilic cytoplasm and round/oval hyperchromatic nuclei, eccentric nucleoli (if present); may appear plasmacytoid, may have significant pleomorphism; sparse fibrous or myxoid stroma; no/rare mitotic figures, no necrosis
Rarely has neuroendocrine differentiation morphologically and immunohistochemically
Positive stains: keratin, mucin (intraluminal), lysozyme, often neuron specific enolase
Negative stains: PAS, chromogranin (usually), synaptophysin (usually), S100, desmin, actin, vimentin
EM: desmosomes and microvilli; often membrane bound dense core granules
DD: jugulotympanic paraganglioma, meningioma, acoustic neuroma, glandular metaplasia (focal or haphazard, in background of chronic otitis media), middle ear adenocarcinoma (marked pleomorphism, mitotic activity, necrosis, invasion of bone and soft tissue)
References: Mod Path 2002;15:543 (adenoma vs. carcinoid tumor)
Middle ear, inner ear and temporal bone tumors-malignant
Extremely rare; arises from middle ear mucosa
Not associated with chronic otitis media
May fill middle ear space and encase ossicles
Symptoms: chronic progressive hearing loss and unilateral otorrhea
Tumor may perforate tympanic membrane and present as external auditory canal mass
Slow growing, locally aggressive, don’t metastasize but may cause death by intracranial extension
Treatment: complete excision
Micro: resemble adenomas but with increased pleomorphism and mitotic figures and extensive infiltration of surrounding structures
DD: metastases (need good clinical history to rule out)
Chondrosarcoma of temporal bone
Rare; most commonly affects petrous apex and posteromedial aspect of temporal bone
Patients may have long survival
Endolymphatic sac papillary tumor
Also called adenoma of endolymphatic sac, adenocarcinoma of temporal bone/mastoid, low grade adenocarcinoma of probable endolymphatic sac origin, papillary adenoma of temporal bone, aggressive papillary tumor of temporal bone, aggressive papillary middle ear tumor, Heffner tumor
Uncommon, associated with von Hippel-Lindau syndrome (11% have these tumors) and female adnexal tumor of presumed wolffian origin (AJSP 1994;18:1254)
Median age 30’s, range of 11-71 years
Symptoms: early sensorineural hearing loss, tinnitus and episodic vertigo
Xray: radiologic features of tumor in posterior-medial petrous ridge of temporal bone (site of endolymphatic sac)
Treatment: radical surgery including mastoidectomy and temporal bone resection, with possible loss of cranial nerves; local recurrence with inadequate surgery
Tumor grows slowly and doesn’t metastasize, but is infiltrative and destructive and may cause death; bleeds profusely at surgery
Case reports: 42 year old woman with tumor and von Hippel Lindau disease (Archives 2003;127:1387), 34 year old Japanese man without von Hippel-Lindau syndrome but with a VHL gene mutation (Hum Path 2001;32:1272); 77 year old man with partially cystic tumor and aspirated cyst fluid analyzed by cytology (Mod Path 2001;14:920); 20 year old woman with von Hippel-Lindau syndrome (Mod Path 2001;14:727); two cases with extensive bone destruction (Mod Path 1995;8:603)
Micro: simple papillary structures composed of single layer of columnar to cuboidal epithelium, often with distinct cell boundaries; may have apparent myoepithelial layer that actually is flattened stroma; epithelial cells have pale-clear cytoplasm, uniform central or luminal nuclei; granulation tissue reaction with small vascular spaces and mixed inflammatory infiltrate is often present next to tumor cells; occasional thyroid-like hypercellular areas with cystic glandular spaces containing colloid-like material; may have areas of recent hemorrhage with cholesterol clefts; minimal pleomorphism; no/rare mitotic figures or necrosis
Cytology: rare epithelial cell clusters, some with papillary features, foamy macrophages; epithelial cells have eosinophilic and focally vacuolated cytoplasm, some with pigmented granules resembling hemosiderin, well defined cell borders, bland nuclei
Positive stains: keratin (diffuse), PAS+, diastase resistant intracytoplasmic material (glycogen), iron; also vascular endothelial growth factor (as with other VHL+ tumors); variable EMA, S100, vimentin, NSE, GFAP, synaptophysin and Leu7
Negative stains: mucin, thyroglobulin, CK20, CEA, inhibin A
EM: intercellular junctional complexes, microvilli, basement membrane, rough ER, glycogen, secretory granules
DD: middle ear adenoma (not papillary, doesn’t invade or destroy bone), choroid plexus papilloma-carcinoma (originates within brain ventricles, S100+), metastatic thyroid carcinoma (thyroglobulin+, characteristic nuclear features), metastatic renal cell carcinoma (S100-, GFAP-, synaptophysin-, kidney tumor present on CT), jugulotympanic paraganglioma (vascular, but not papillary-cystic, has “Zellballen” nesting pattern, keratin negative)
References: AJSP 1988;12:790, Cancer1989;64:2292
Case report of temporal bone tumor (Archives 2003;127:1217)
Clonal proliferation of Langerhans cells, either isolated or as part of systemic process
Usually males, teens to twenties
Common sites are middle ear and temporal bone
Xray: single or multiple sharply circumscribed osteolytic lesions
Treatment: excision (curettage) or low dose radiation therapy; good prognosis, cure if no recurrence within 1 year; chemotherapy if systemic
Micro: sheets, nests or clusters of Langerhans cells, which have moderate eosinophilic cytoplasm, bean-shaped nuclei with indentations, vesicular chromatin with small nucleoli; also neutrophils, plasma cells, lymphocytes, variable foamy histiocytes and multinucleated giant cells; no/mild tumor cell pleomorphism, no/rare mitotic figures
Positive stains: Langerhans cells - S100, CD1a; histiocytes and multinucleated cells - CD68
EM: Birbeck granules
DD: sinus histiocytosis with massive lymphadenopathy (CD1a negative), non-Hodgkin lymphoma (CD20+, S100-, CD1a-)
Usually from breast, lung, kidney; also prostate and melanoma, but almost all sites have been described
Also direct extension from tumors of pharynx, salivary gland, central nervous system
Often associated with Paget’s disease of bone, fibrous dysplasia or radiation therapy
Aggressive, often metastasize to lung and brain
5 year survival: <15%
Usually a disease of children; no gender preference
Painless unilateral otitis media unresponsive to antibiotics
Often has invaded external canal, mastoid and meninges at presentation
International classification: I-superior prognosis (botyroid and spindle cell types); II-intermediate prognosis (embryonal type); III-poor prognosis (alveolar and undifferentiated types); IV-unknown prognosis (rhabdoid features)
Poor prognostic features: meningeal involvement; subtypes above
May metastasize to local lymph nodes, lung or bones
5 year survival: 74% for pediatric patients
Treatment: surgery, radiation therapy, chemotherapy
Gross: polypoid lesion of external or middle ear
Micro: usually embryonal or botyroid types (neoplastic tumor cells growing beneath a flattened epithelium; usually small cells but occasional large cells with eosinophilic, fibrillary cytoplasm); alveolar is less common; other types are rare
DD: inflammatory polyp / granulation tissue
Rare; usually age 50’s and 60’s
Usually >20 year history of chronic otitis media or radiation therapy for intracranial tumors or middle ear inflammation
25% have associated cholesteatomas
Symptoms: long standing chronic otitis media with sudden onset of pain, otorrhea, hemorrhage
Treatment: radical surgery with radiation therapy, chemotherapy for advanced disease
5 year survival: 39%; 10 year survival: 21%; usually no metastatic disease
Gross: tumor fills middle ear spaces, may invade walls of mastoid air cells, bone adjacent to carotid canal, internal auditory meatus, Eustachian tube, external auditory canal
Micro: resembles squamous cell carcinomas at other sites; well to poorly differentiated; infiltrative malignant cells with keratinization, intercellular bridges
DD: cholesteatoma (no atypia), metastases (need good history to rule out), direct invasion from nasopharynx, skin, external ear, parotid gland
Miscellaneous
External ear carcinomas and melanomas are staged the same as other skin carcinomas and melanomas
Orientation is essential; may need to consult with surgeon, particularly if middle ear or inner ear tissue
Must determine surgical resection margins
Extensive decalcification may be needed
Stapes: orient for embedding after decalcification to reveal outline of entire ossicle
End of Ear chapter/outline