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Table of contents
Primary references, normal anatomy, normal histology
Congenital anomalies: general, accessory tragi, first branchial cleft anomalies, heterotopia/choristoma
Inflammatory/infectious/autoimmune/systemic disorders: angiolymphoid hyperplasia with eosinophilia, chondrodermatitis nodularis chronica helicis, gout, granuloma inguinale, idiopathic auricular ossificans, idiopathic cystic chondromalacia of auricular cartilage, inflammatory aural/otic polyp, Kimura’s disease, labyrinthitis, malakoplakia, Meniere’s disease, myospherulosis, necrotizing “malignant” external otitis, otitis media, otomycosis, otosclerosis, Paget’s disease, pneumocystis, presbycusis, relapsing polychondritis, Wegener’s granulomatosis
External ear tumors-benign/non-neoplastic: amyloid nodules, atypical fibroxanthoma, ceruminal gland adenoma, cholesteatoma, collagenous papules, elastotic nodules, exostosis, keloid, keratinous cyst, keratoacanthoma, keratosis obturans, myofibromatosis, myxoma, neurothekeoma, osteoma, solitary fibrous tumor, synovial chondromatosis
External ear tumors-malignant: basal cell carcinoma, ceruminal gland adenocarcinoma, malignant blue nevus, melanoma, metastases, squamous cell carcinoma
Middle ear, inner ear and temporal bone tumors-benign/non-neoplastic: acoustic neuroma, cholesteatoma, chondroblastoma, jugulotympanic paraganglioma, lipochoristoma, lipoma, meningioma, middle ear adenoma, papilloma
Middle ear, inner ear and temporal bone tumors-malignant: adenocarcinoma, aggressive papillary tumor, chondrosarcoma, giant cell tumor, Langerhans cell histiocytosis, lymphoepithelioma-like carcinoma, malignant peripheral nerve sheath tumor, metastases, osteosarcoma of skull, rhabdomyosarcoma, squamous cell carcinoma
Miscellaneous: TNM staging, grossing
Primary references for Ear chapter
American Journal of Clinical Pathology (AJCP), May 1975 to December 2006
American Journal of Surgical Pathology (AJSP), March 1977 to December 2006
Archives of Pathology and Laboratory Medicine (Archives), January 1976 to October 2006
Human Pathology (Hum Path), March 1970 to November 2006
Journal of Clinical Pathology (J Clin Path), January 1975 to November 2006
Modern Pathology (Mod Path), January 1988 to December 2006
Rosai, J: Ackerman’s Surgical Pathology (9th Ed); Mosby, 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
Normal anatomy of ear
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
Drawings: lateral surface of pinna #1; #2; external and middle ear #1; #2; #3; external, middle and inner ear #1; #2; tympanic membrane
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, a 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
Drawings: tympanic membrane; inner wall of middle ear #1; #2; eustachian tube; chain of ossicles #1; #2; malleus; incus; stapes; temporal bone
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), and internal auditory canal (contains vestibulocochlear nerve, CN VIII)
Vestibular labyrinth contains semicircular canals, 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
Drawings: labyrinth #1; #2; #3; #4; cochlea and surrounding structures; cochlea cross section; floor of cochlea; spiral organ of Corti #1; #2; semicircular canal cross section; endolymphatic sac (see inset); endolymphatic sac (ES) and duct (ED)
References: Wikipedia (cochlea)
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
Micro images: elastic cartilage (elastic stain)
Virtual slides: pinna of ear
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
Micro images: cochlea; hair cells (mammalian)
Congenital anomalies of ear
Congenital anomalies of ear-general
Common (1 per 6000 births); higher incidence in Japanese and Navajo Indians
More common in males
90% are unilateral
Either isolated or with other abnormalities
Cosmetic or functional
Anotia: complete absence of external ear
Microtia: mild to severe deformity; associated with other defects
Also called accessory or supernumerary ear, accessory auricle, polyotia
Appears at birth; incidence of 0.2 to 0.5%
May be related to second branchial arch anomalies
Usually no associated hearing loss or anomalies (Int J Pediatr Otorhinolaryngol 2002;63:25)
Associated in some cases with cleft lip/palate, mandibular hypoplasia, oculoauriculovertebral dysplasia (Goldenhar syndrome, OMIM 164210)
Solitary or multiple, unilateral or bilateral, sessile or pedunculated, soft or cartilaginous
Treatment: simple excision
Gross: skin covered nodule, often anterior to auricle
Micro: skin with cutaneous adnexae and central cartilage (resembles normal external auricle)
Micro images: various images
DD: squamous papillomas (no cartilage, no adnexae)
First branchial cleft anomalies of ear
Cysts, sinuses, abscesses (Emerg Med J 2003;20:103), and fistulas near external ear; may be pre-, post- or infraauricular; also at angle of jaw, ear lobe, in external auditory canal or parotid gland
Fistulas may connect skin with external auditory canal
Includes duplication of external auditory canal (Int J Pediatr Otorhinolaryngol 2005;69:255)
1-8% of branchial defects
Usually middle aged women
Classified by Work as Type I or II (Laryngoscope 1972;82:1581)
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
References: branchial anomalies
Heterotopia / choristoma of ear
See also Lipochoristoma
WHO classification includes salivary gland choristoma and glial choristoma
Heterotopia: mass of tissue normal to the site in abnormal location
Choristoma: mass of tissue foreign to the site
Hamartoma: mass of tissue normal to site in haphazard arrangement
Middle ear choristomas include salivary gland tissue and neuroglial tissue
Salivary gland choristomas usually occur in women, associated with facial nerve and ossicle anomalies, suggesting a second branchial arch developmental anomaly; have mucinous and serous elements similar to submandibular and sublingual glands
Neuroglial choristomas are often actually an acquired encephalocele with herniation of brain into middle ear and mastoid; treatment is surgical, although tissue may adhere to facial nerve; determine relationship to CNS structures at operation or radiographically, not by histology (Laryngoscope 2000;110:1731, Ann Diagn Pathol 2004;8:252)
Cartilaginous choristoma of external ear canal: may be relatively common (Otolaryngol Head Neck Surg 2005;133:786)
Case reports: salivary gland tumors of middle ear (Archives 1982;106:39, Laryngoscope 2006;116:1033), glial choristoma of middle ear and mastoid bone (J Korean Med Sci 2004;19:155)
References: salivary gland choristomas of middle ear (ORL J Otorhinolaryngol Relat Spec 2004;66:141)
Inflammatory/infectious/autoimmune/systemic disorders of ear
Angiolymphoid hyperplasia with eosinophilia of ear
Also called epithelioid (histiocytoid) hemangioma
Rare, 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
Case reports: auricular nodule during pregnancy (Archives 2005;129:1168), regression after excision of squamous cell carcinoma on same ear (Dermatol Surg 2004;30:1367), 20 year history of lesions (Dermatol Online J 2002;8:10)
Treatment: local excision or laser desiccation are usually curative
Gross: pink-red-brown cutaneous papules or subcutaneous nodules up to 1 cm; may coalesce to form plaque-like lesions
Gross images: papule on ear
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
Cytology: no evidence of malignancy; vascular structures, eosinophils, lymphocytes, and clusters of cuboidal cells with vacuoles in abundant acidophilic cytoplasm (Diagn Cytopathol 1998;18:227)
Micro images: various images #1; #2; thick walled vessels lined by prominent epithelioid cells with inflammatory infiltrate
DD: hemangioma (no epithelioid endothelial cells, no inflammatory infiltrate), angiosarcoma (anastomosing vascular channels lined by pleomorphic cells with increased mitotic activity, no inflammatory infiltrate), Kimura’s disease (large, deep, subcutaneous plaques in young Asian men, often regional lymphadenopathy, peripheral blood eosinophilia and elevated serum IgE levels; prominent lymphoid follicles and fibrosis with less prominent capillary proliferation, no aggregates of non-canalized endothelial cells)
References: eMedicine #30
Chondrodermatitis nodularis chronica helicis
Also called Winkler’s disease
Idiopathic, nonneoplastic ulcerative lesion of auricle
Usually men ages 40+; uncommon in women
Symptoms: appears spontaneously; unilateral, painful nodule
Treatment: wedge or cartilage excision, glucocorticoid injection; frequently recurs
Gross: dome shaped nodule, 0.3 to 1.8 cm, with crusty scale covering central area of ulceration
Gross images: various nodules
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; usually nerve hyperplasia identified with S100 (J Am Acad Dermatol 2006;55:844); may have foci of fibrinoid necrosis; no dermal adnexa at site of lesion
Micro images: infiltration of papillary dermis by epithelioid (E) cells; various images #1; #2
DD: clinically resembles carcinoma; vascular proliferation may resemble a glomus tumor
References: J Clin Path 1959;12:179, eMedicine
Primary gout (90%): idiopathic (85%) due to overproduction of uric acid or known enzyme defects (5%, partial hypoxanthine-guanine phosphoribosyl transferase deficiency [HGPRT]); may have normal excretion
Secondary gout (10%): increased nucleic acid turnover due to leukemia/lymphoma, chronic renal disease, HGPRT deficiency
See also Joints chapter
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 nodules
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
Micro images: site unknown - A: Xray; B: Diff-Quick; C: Pap smear; D: polarized light; E: H&Exf
DD: pseudogout (rhomboid or needle shaped, weak positive birefringence with polarized light, radiographic calcifications)
References: Wikipedia
Also called donovanosis
Rare in children and at this site; may be transmitted during vaginal delivery
Case reports: children 5 and 8 months old with mastoiditis and external ear discharges (AJCP 1997;108:510)
Treatment: antibiotics
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)
References: J Am Acad Dermatol 2003;49:142, Laryngoscope 1985;95:566
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
Case reports: 32 year old man with auricular swelling (J Clin Path 1994;47:961)
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
Micro images: central cystic degeneration of auricular cartilage; factor VIII+ lining of pseudocyst
DD: relapsing polychondritis, subperichondrial hematoma, chondrodermatitis nodularis helicis chronicus
References: Archives 1986;110:740, Eur J Dermatol 2000;10:451, eMedicine
Inflammatory aural / otic 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
Often associated with extensive disease of mastoid air cell system (in rural India, Am J Otolaryngol 2003;24:155) or cholesteatoma of middle ear (Acta Otorrinolaringol Esp 2003;54:161)
Treatment: excision; possibly mastoid exploration
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; usually mast cells (J Laryngol Otol 1995;109:491), variable neutrophils, multinucleated giant cells, cholesterol granulomas and tympanosclerosis
DD: plasmacytoma (monoclonal light chains)
Asians, usually males
Large subcutaneous nodules, usually in head and neck, with regional lymphadenopathy and peripheral eosinophilia
Case reports: with coexisting angiolymphoid hyperplasia with eosinophilia (Int J Dermatol 2006;45:139), with bilateral auricular masses (AJNR Am J Neuroradiol 1999;20:1976)
Micro: prominent lymphoid proliferation, sparse vascular proliferation; often extends to fascia and skeletal muscle; adipose tissue is often fibrotic; eosinophils are common
Micro images - neck mass - nodular, mixed inflammatory infiltrate of reticular dermis; infiltrate is poorly circumscribed and contains several lymphoid follicles; lymphoid hyperplasia; dense inflammatory infiltrate with numerous eosinophils
DD: angiolymphoid hyperplasia with eosinophils (more superficial, may have rare/no eosinophils), angiosarcoma
Inflammation of inner ear secondary to various causes
Rare today due to antibiotics for otitis media, syphilis and other diseases
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), due to acute otitis media (Yonsei Med J 2005;46:161)
Micro images: purulent labyrinthitis
References: eMedicine #1, #2, Wikipedia
Malakoplakia of ear
Rarely occurs in middle ear
Due to inability of histiocytes to ingest bacteria such as E coli, with accumulation in phagolysosomes
Case reports: 9 month old with involvement of temporal bone (J Laryngol Otol 1991;105:568)
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)
Micro images: various images-not ear
Positive stains: Michaelis-Gutmann bodies are PAS+ diastase resistant, Prussian blue/iron+, von Kassa/calcium+
Also called endolymphatic hydrops
Idiopathic disorder of inner ear associated with episodic attacks of vertigo, fluctuating 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
Rarely occurs in children (J Laryngol Otol 2006;120:343)
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; 60-80% improve; surgery includes shunting and decompression of endolymphatic sac (Laryngoscope 2005;115:1454), 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
Micro images: section through cochlea
References: National Institutes of Health, Wikipedia, eMedicine
Iatrogenic due to petrolatum based ointments
Usually nasal cavity and paranasal sinuses; occasionally affects middle ear
History of recent surgery with packing of area (AJCP 1977;67:118)
Associated with postoperative adhesions after sinus surgery (Laryngoscope 2003;113:1123)
Case reports: post-tympanoplasty (Laryngorhinootologie 2004;83:445); involving mastoid and middle ear (Laryngorhinootologie 2004;83:445)
Treatment: symptomatic
Gross: ointment-like material within granulation tissue
Micro: pseudocysts within fibrous tissue with lymphocytes, histiocytes, giant cells and plasma cells; pseudocysts contain “parent bodies” containing numerous spherules
Micro images: Aspergillus (figure 3) and myospherulosis (figure 4) in sinus
Negative stains: GMS for fungi
Necrotizing “malignant” external otitis
Potentially fatal external otitis due to Pseudomonas aeruginosa (Ann Otolaryngol Chir Cervicofac 2000;117:291), Aspergillus or other fungal infection
Usually older patients, often with diabetes, chronic debilitation or immunodeficiency; also undernourished African infants (Rev Laryngol Otol Rhinol (Bord) 2002;123:225)
Initially affects external auditory canal with symptoms of acute otitis externa; later pain, purulent otorrhea and swelling; may progress to cellulitis, chondritis, osteomyelitis (Rev Stomatol Chir Maxillofac 2006;107:167), 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 above primary pathologic state) plus neutrophilic migratory defect plus virulence of Pseudomonas
Treatment: antibiotics, surgical debridement, hyperbaric oxygen (HNO 2003;51:315)
Case reports: oxalate crystals within necrotic tissue are associated with Aspergillus niger infection (Mod Path 1993;6:493), due to Klebsiella pneumoniae infection (Eur Arch Otorhinolaryngol 2006;263:344), Stenotrophomonas maltophilia (Hautarzt 2003;54:1080), Staphylococcus epidermidis (Eur Arch Otorhinolaryngol 1999;256:439)
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
References: American Family Physician
Acute or chronic infectious disease of middle ear
Usually childhood disease caused by Streptococcus pneumoniae or Haemophilus influenzae (Pediatr Infect Dis J 2004;23:1142); also coinfection by viruses (Clin Infect Dis 2006;43:1417)
Rarely caused by fungi or Pneumocystis in HIV+ patients
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
Treatment: antibiotics (Pediatr Infect Dis J 2006;25:1102) or observation (Lancet 2006;368:1429); 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 (Laryngoscope 1982;92:273) 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
Micro images: serous otitis media
DD: middle ear adenoma (regular, not haphazard glands, no cilia)
References: eMedicine #1, #2
Micro images: contributed by Professor Venna Maheshwar, Drs. Kiran Alam and Anshu Jain, J. N. Medical College, India - #1; #2; #3
Disorder of bone remodeling affecting bony labyrinth and stapes footplate
Does not appear to occur outside temporal bone (Am J Otol 1999;20:162)
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
Associated with measles virus (Ann Otol Rhinol Laryngol 2001;110:897)
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
Micro images: a. Temporal bone with otosclerosis fixing the stapes; b. Otosclerosis involving the wall of the cochlea; c. Normal Bone
References: Wikipedia, eMedicine
Paget’s disease of bone of temporal bone
Also called osteitis deformans
Chronic progressive disease of increased bone turnover, affects skull and temporal bone in 70% of cases, with hearing loss in half of these cases
Hearing loss is due to compression of cranial nerve VIII and apparently loss of mineral density of cochlear capsule (Laryngoscope 2004;114:598)
Familial cases are due to mutations in bone metabolism pathway genes (Nat Clin Pract Rheumatol 2006;2:270)
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
Micro images - not necessarily temporal bone - multiple osteoclasts; increased cement lines
DD: otosclerosis (younger age, doesn’t affect skull)
References: eMedicine, OMIM 602080
Case reports: HIV+ patient with infection of middle ear #1 (Archives 1992;116:500), #2 (ORL J Otorhinolaryngol Relat Spec 2003;65:49); presenting as bilateral aural polyps in HIV+ patient with no prior Pneumocystis (J Laryngol Otol 2002;116:288)
Hearing loss associated with aging, often due to degeneration of cochlear hair cells or spiral ganglion cells
Usually no surgical specimen
References: eMedicine, National Institutes of Health
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
Earlobes are typically spared
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
Gross images: diffuse erythema sparing earlobe
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)
References: Clin Dermatol 2006;24:482; eMedicine
Wegener’s granulomatosis of ear
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); extra levels/recuts increases sensitivity by 7% (Ann Path 2005;25:87)
Micro images - granulomatous vasculitis
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)
References: Reumatismo 2005;57:187, eMedicine, Wikipedia
External ear tumors-benign/non-neoplastic
Amyloid nodules of external ear
Slightly friable but not pruritic
Usually no other evidence of amyloidosis
Gross: small papules on concha of ear
Micro: amorphous material
Positive stains: cytokeratin 34betaE12, EKH4 keratin (J Am Acad Dermatol 1988;18:19)
DD: collagenous papules of ear
Atypical fibroxanthoma of external ear
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
Case reports: clear cell variant (J Cutan Path 2006;33:343), granular cell variant (Pathol Oncol Res 1996;2:244)
Treatment: excellent prognosis with complete excision; 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
Micro images: forehead mass
Positive stains: vimentin, CD68, CD99; variable actin
Negative stains: keratin, S100, HMB45, desmin
DD: spindle cell carcinoma, spindle cell melanoma, leiomyosarcoma (desmin+), malignant fibrous histiocytoma (> 2 cm, extensively infiltrative and either necrosis or vascular invasion)
Ceruminal gland adenoma of external auditory canal
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
Term “ceruminoma” is obsolete
Symptoms: slow growing external auditory canal mass or blockage with conductive hearing loss
Case reports: expansion into middle ear (Laryngorhinootologie 2006;85:444)
Treatment: complete surgical excision; recurrences are due to incomplete excision
Gross: skin covered, circumscribed, polypoid or rounded mass, 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
Micro images: thick, apparently bilayered epithelium forming ductal structures with many papillations and decapitation apocrine secretions
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 of external auditory canal
Uncommon
Case reports: lipomatous variant (Pathol Int 2006;56:51), extensive apocrine glandular component (Acta Otorhinolaryngol Ital 2002;22:158), apocrine differentiation and fat cells (Pathol Int 1994;44:80); 4 mm mass of middle ear
Micro: resembles salivary gland tumor with mixture of epithelial and myoepithelial cells in myxoid stroma
Micro images: epithelial glands and myoepithelial cells; keratin+ (top); S100+ (bottom)
Cytology: typical findings of pleomorphic adenoma (Acta Cytol 1999;43:489)
References: AJSP 2004;28:308
Syringocystadenoma papilliferum of external auditory canal
Rare in ear
Benign tumor of apocrine gland origin
Usually scalp or face, but also external auditory canal
Case reports: tumor of external auditory canal (J Laryngol Otol 2005;119:1004), treated with Moh’s surgery (Dermatol Surg 2004;30:468)
Micro: similar to tumor at other sites - papillary architecture with marked plasma cell infiltrate
Micro images: various images
Cholesteatoma of external auditory canal
Rare; differs from middle ear cholesteatoma
May extend into mastoid or middle ear, may involve facial nerve canal or tegmen tympani (AJNR Am J Neuroradiol 2003;24:751)
Staging criteria at Laryngoscope 2005;115:455
Case reports: extensive invasion of mastoid (Rev Bras Otorrinolaringol 2005;71:91), with osteoid osteoma (Auris Nasus Larynx 2005;32:281)
Micro: cystic mass of keratinized squamous epithelium overlying area of bone sequestration in external auditory canal
DD: keratosis obturans (no osteonecrosis, no focal overlying epithelial loss, Clin Otolaryngol Allied Sci 2004;29:577)
References: University of Texas Medical Branch
Collagenous papules of external auditory canal
Bilateral, smooth, firm, small papules on inner aspects of pinna
Rarely involves external auditory canal
Some of these cases may represent amyloid
Micro: dense collagen with dilated vessels and scattered fibroblasts
References: Am J Dermatopathol 1983;5:231
Elastotic nodules of external auditory canal
Small papules and nodules, often on antihelix
Due to actinic damage
Case reports: bilateral nodules (Cutis 1989;44:452)
Micro: dermal clumps of elastic tissue
References: J Cutan Pathol 1981;8:429
Exostosis of external auditory canal
Also called surfer’s ear
Reactive, localized overgrowth of bone, with a broad base
Called osteoma if pedunculated
Arises 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 (J Laryngol Otol 2004;118:348)
Treatment: medical (for external otitis-antibiotics and aspiration), transmeatal surgical excision if medical treatment fails and patient is symptomatic (ORL J Otorhinolaryngol Relat Spec 2003;65:189)
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)
References: Wikipedia
Keloid of external auditory canal
Greek for “crab claw”
Exaggerated, non-neoplastic scarring response to trauma
Common in young black women after ear piercing
Case reports: at site of prior meatoplasty (J Laryngol Otol 2006;120:594)
Treatment: excision (although 40% recur), intralesional steroids or interferon, shave excision plus imiquimod cream (Dermatol Surg 2006;32:380), possibly shaving plus cryosurgery (Ann Dermatol Venereol 2006;133:225), possibly radiotherapy (J Med Assoc Thai 2006;89:428)
Gross: polypoid, covered by thin, hairless skin; usually < 2 cm
Gross images: on pierced ear
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
Micro images: site unknown - various images; low power #1; #2
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)
References: eMedicine
Keratinous cyst of external auditory canal
Common around external ear
Related to branchial cleft or epidermal inclusion cysts
Recurrence rate of 13% with punch incision technique (Dermatol Surg 2002;28:673)
Case reports: pigmented epidermal cyst (J Dermatol 1997;24:475)
Micro: lined by keratinized squamous epithelium, filled with keratin
Micro images: site unknown - epidermal inclusion cyst
Keratoacanthoma of external auditory canal
Common in skin of external ear
Often rapid growth (days/weeks, HNO 1993;41:532)
Case reports: giant keratoacanthoma in 84 year old Japanese woman (Auris Nasus Larynx 2000;27:185)
Micro images: site unknown - keratoacanthoma
DD: squamous cell carcinoma
Keratosis obturans of external auditory canal
Accumulation of keratin debris deep within external auditory canal, which may cause bone remodeling and inflamed epithelium
May cause severe erosion of petrous bone (Br J Radiol 1975;48:170)
Case reports: extensive erosion of the hypotympanum with exposure of the facial nerve and the annulus of the tympanic membrane (J Laryngol Otol 2003;117:725)
Treatment: removal of keratin plug; surgery not required
Gross images: keratin debris with cerumen
Micro: tightly packed keratin squames in lamellar pattern; diffuse acanthosis and hyperkeratosis of skin of canal with underlying chronic inflammatory infiltrate; no osteonecrosis, no overlying epithelial loss
DD: cholesteatoma (Clin Otolaryngol Allied Sci 2004;29:577, Arch Otolaryngol 1984;110:690)
Myofibromatosis of external auditory canal
Usually children and neonates; may be multicentric
Benign tumor of skin or superficial soft tissue
Excellent prognosis, often involutes spontaneously
Case reports: solitary tumor in ear canal of adult (AJCP 1992;97:810); in pinna of 50 year old woman (J Laryngol Otol 1999;113:155)
Micro images: infantile myofibromatosis (similar histology) - various images; axilla - various images
Myxoma of external auditory canal
May arise as part of Carney complex (Semin Dermatol 1995;14:90)
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)
Case reports: 17 year old boy with tumor of temporal bone (Neurosurgery 2001;48:945)
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; may have pseudocapsule of condensed reticulin fibers and compressed skeletal muscle; no pleomorphism
Micro images: site unknown - myxoma #1; #2
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)
Neurothekeoma of external auditory canal
Also called peripheral nerve sheath myxoma
Case reports: isolated tumor in lateral external auditory canal (J Otolaryngol 1989;18:90)
Osteoma of external auditory canal, middle and inner ear
True neoplasms capable of unlimited growth, in contrast to exostosis
Common in ancient populations in middle ear, particularly Bedouins (Acta Otolaryngol 2005;125:1164)
Asymptomatic solitary masses attached by narrow pedicle to tympanosquamous or tympanomastoid suture line
Case reports: with cholesteatoma (Auris Nasus Larynx 2005;32:281), huge osteoma of middle ear (Int J Pediatr Otorhinolaryngol 2005;69:1569), osteomas of internal auditory canal (Am J Otol 2000;21:852)
Micro: mature bone with bone marrow and intraosseous fibrovascular tissue covered by keratinized squamous epithelium
Micro images: site unknown - osteoma #1; #2
Solitary fibrous tumor of external auditory canal
Slow growing tumor, usually benign, although malignant transformation and metastases have been reported at other sites
Very rare in auditory canal
Case reports: 39 year old woman with tumor of auditory canal (Archives 2004;128:e169), 38 year old man with tumor at entrance of external auditory meatus (Auris Nasus Larynx 2004;31:65)
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
Micro images: figure 2a: spindle cell lesion; 2b: fascicular/whorled patterns; 2c: vascular clefts; 2d: CD34+; 2e: CD99+; 2f: bcl2+
other sites - kidney #1; #2; #3; #4-CD34
Positive stains: vimentin, CD34, bcl2, CD99
Negative stains: keratin, EMA, S100, CD31, neurofilament
Synovial chondromatosis of temporomandibular joint / external auditory canal
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 (Arch Otolaryngol Head Neck Surg 1999;125:1394)
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
Gross images: site unknown - numerous nodules #1; #2
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
Micro images: site unknown - low power #1; high power #1; #2
DD: chondrosarcoma
External ear tumors-malignant
Basal cell carcinoma of external auditory canal
Common tumor of auricle, but rare in external auditory canal
Untreated tumors may extend into middle ear, mastoid or cranial cavity
Even treated tumors of external auditory canal have aggressive behavior (Acta Chir Belg 2002;102:137)
Case reports: auricular tumor with metastases to parotid gland (Dermatol Online J 2006;12:7), external auditory canal tumor with nevoid basal cell carcinoma (Gorlin-Goltz) syndrome (J Laryngol Otol 1997;111:850)
Treatment: surgery, radiation therapy
Micro images: site unknown - various images
Ceruminal gland adenocarcinoma of external auditory canal
Usually men, ages 30-59 years but wide age range
Associated with local pain
Tends to recur locally; only rarely metastasizes to regional lymph nodes and lung
Adenoid cystic carcinoma subtypes (Eur Arch Otorhinolaryngol 1993;250:240) 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 contralateral brain metastasis (Archives 2002;126:87), causing death (Acta Morphol Hung 1991;39:157), with recurrence (ORL J Otorhinolaryngol Relat Spec 2003;65:300), invading temporal bone (Cancer 1978;41:545)
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
Malignant blue nevus of external auditory canal
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)
DD: deep penetrating nevi (Arch Dermatol 2003;139:1608)
Melanoma of outer ear / external auditory canal
Either external ear (1% of US melanoma cases) or external auditory canal (rare)
External ear
Usually auricle
Usually superficial spreading type, affecting anterior helix
43% develop recurrence or metastases (Ann Plast Surg 2006;57:70)
Poor prognostic factors: ulceration, thick tumor, high stage (Br J Dermatol 2006;154:310)
Gross images: melanoma of external ear
External auditory canal
Often silent and aggressive (Acta Otorrinolaringol Esp 2003;54:89, Am J Clin Oncol 1998;21:28)
Metastases to external auditory canal
Case reports: colon cancer metastases causing hearing loss (Ear Nose Throat J 2005;84:36)
Squamous cell carcinoma of outer ear / external auditory canal
15% of primary cutaneous carcinomas of external ear and auditory canal
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
University of Pittsburgh staging system: Am J Otol 2000;21:582
Case reports: squamous cell carcinoma in situ of external auditory canal (J Laryngol Otol 2006;120:684), bilateral tumors (HNO 2006;54:41, Laryngoscope 2002;112:1003), with angiolymphoid hyperplasia (Dermatol Surg 2004;30:1367), pigmented with dendritic melanocyte colonization (Path Int 1999;49:909)
Treatment: complete excision (mastoidectomy or temporal bone resection for canal tumors), possibly radiation therapy
Tumors of external ear have low recurrence rate (Dermatol Surg 2005;31:1423)
Canal tumors often recur (19%) or metastasize (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
Contributed by Dr. Semir Vranic: angiolymphatic invasion - #1; #2
site unknown - various images
DD: irritated seborrheic keratosis, pseudoepitheliomatous hyperplasia (Laryngoscope 1998;108:620), melanoma, malignant fibrous histiocytoma,
References: Auris Nasus Larynx 2006;33:251
Spindle cell carcinoma of external auditory canal
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 carcinoma of external auditory canal
Unusual variant
Also called pseudoglandular or acantholytic
Often face and scalp in sun-exposed areas, particularly periauricular area
Due to a desmosomal defect that causes lack of cell adhesion (acantholysis)
Micro: pseudoglandular appearance due to tumor cell acantholysis in center of tumor nests; usually dysplastic surface epithelium
Negative stains: mucin
DD: adenocarcinoma, vascular tumors, adenosquamous carcinoma
Middle ear, inner ear and temporal bone tumors-benign/non-neoplastic
Acoustic neuroma of middle/inner ear or temporal bone
Common terminology but inaccurate because does not derive from acoustic branch of CN VIII, and is not a neuroma
Also called vestibular schwannoma, neurilemmoma, benign peripheral nerve sheath tumor
Benign (rarely malignant if associated with neurofibromatosis) 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 tumors as teenagers
Xray: flaring, widening or erosion of internal auditory canal
Case reports: schwannoma of tympanic membrane (J Laryngol Otol 2006;120:247). arising from Jacobsen’s nerve of middle ear (AJNR Am J Neuroradiol 2000;21:1331)
Treatment: complete excision, although often slow growing and may not need surgery (Skull Base 2006;16:95)
Gross: circumscribed, tan-white-yellow, rubbery/firm, up to 5 cm, variable cystic change
Gross images: tumor in cerebellopontine angle
Micro: unencapsulated but otherwise resembles schwannomas at other locations; interlacing fascicles of cells with indistinct cytoplasmic borders, elongated and twisted nuclei; biphasic with Antoni A (cellular) and B (hypocellular, myxoid) patterns and Verocay bodies (whorling or palisading of nuclei); hyalinization of vessels; commonly have regressive (“ancient”) changes (cellular pleomorphism with hyperchromasia, cystic degeneration, necrosis, calcification, hemorrhage); may be highly cellular (cellular schwannoma); no/rare mitotic figures
Cystic change may be due to intratumoral hemorrhage (Neurosurg 2006;105:576)
Micro images: interlacing fascicles of Schwann cells; Antoni A pattern; Antoni B pattern; CD57/Leu7
Positive stains: S100 (diffuse, strong)
Negative stains: keratin, chromogranin, synaptophysin
EM images: various images
References: Wikipedia, eMedicine
Cholesteatoma of middle/inner ear or temporal bone
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
Usually men, ages 20’s to 30’s
May be (a) congenital (squamous epithelium is trapped within temporal bone during embryogenesis; tympanic membranes are intact; no history of infections), (b) primary acquired (retraction of tympanic membrane), (c) secondary acquired (due to injury to tympanic membrane)
May cause progressive bone erosion of ossicles and surrounding bone, but is not neoplastic; incomplete excision may lead to widespread bone destruction
Damage apparently is due to associated inflammation and proteolytic enzymes, not pressure from tumor mass (Mod Path 2001;14:1226)
Aural polyps composed of granulation tissue and with keratin flakes or masses are usually associated with underlying cholesteatoma (J Clin Path 1989;42:460)
Prognostic factors: presence of papillary epithelial hyperplasia and marked koilocytosis is associated with aggressive disease and HPV (Med Sci Monit 2006;12:CR368)
Case reports: cerebellar invasion (Archives 1985;109:960)
Treatment: complete excision of all components
Gross: cystic, white masses of varying size with creamy or waxy granular material
Gross images: yellow-white waxy material
Micro: keratinized stratified squamous epithelium (required for diagnosis) with granulation tissue and keratin debris; also chronic inflammatory infiltrate, cholesterol clefts, foreign body giant cell granulomas, hemosiderin; no dysplasia
Micro images: A: matrix, B: perimatrix, C: cystic content; squamous epithelium with keratin debris
DD: squamous cell carcinoma (atypia, desmoplasia)
References: Rev Bras Otorrinolaringol (Engl Ed) 2005;71:536, eMedicine #1, #2
Chondroblastoma of temporal bone
Rare
Often in lateral temporal bone
Usually males
Case reports: with high proliferative activity (Neurol Med Chir (Tokyo) 2002;42:516), 34 year old woman (No To Shinkei 1992;44:143), Otolaryngol Head Neck Surg 1979;87:229
Treatment: wide excision; 50% recur
Micro: clusters of round/polygonal chondroblasts with oval or grooved nuclei and well defined cell borders; also multinucleated giant cells; may have aneurysmal bone cyst-like areas, chondroid matrix
Micro images: figure 4
Positive stains: S100
Negative stains: CD34, CD99
References: AJCP 1987;88:1, J Korean Med Sci 1999;14:559
Jugulotympanic paraganglioma of middle ear
Also called glomus jugulare tumor or glomus tympanicum tumor
Most common tumor of middle ear
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 usually benign, but this does not predict behavior
Rarely are malignant histologically (necrosis, mitotic activity, vascular invasion), with metastases to cervical lymph nodes, lung, liver (J Laryngol Otol 2000;114:17)
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 (zellballen) 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
Micro images: other sites - paraganglioma-bladder #1; #2-S100; site unknown
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
Molecular: germline mutations for succinate dehydrogenase gene subunits if multiple tumors (Diagn Mol Path 2005;14:109)
DD: other neuroendocrine tumors, melanoma, carcinoma, middle ear adenoma, acoustic neuroma, meningioma
References: eMedicine
Lipochoristoma of middle / inner ear or temporal bone
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; associated with hearing loss
Treatment: conservative surgery with nerve preservation
Gross: arise from CN VIII (cochlear or vestibular branch), pink-ivory-gray-maroon, soft-rubbery, unilateral
Micro: normal nerve components (myelinated nerve branches, glia cells, neurons, small thin walled vessels), mature adipose tissue with variable amounts of mature fibrous tissue, tortuous thick walled vessels, smooth muscle bundles and skeletal muscle fibers
References: Archives 2003;127:1475
Lipoma of internal auditory canal
Rare; intracranial lipomas are present in < 0.1% of general autopsies, but only 3% of these are within internal auditory canal
Mean age 40 years but all ages are affected
Often symptomatic; rarely causes eustachian tube dysfunction (J Laryngol Otol 1997;111:1162)
Case reports: 2 cases resembling vestibular schwannoma/acoustic neuroma #1 (Archives 1996;120:681), #2 (Zentralbl Neurochir 2004;65:88), 46 year old woman (Archives 2005;129:1491), with history of multiple lipomas (Ear Nose Throat J 2001;80:340), angiofibrolipoma of external auditory canal (Laryngoscope 2005;115:1461)
Gross: soft, lobulated, yellow tissue infiltrating into adjacent nerves (Otolaryngol Head Neck Surg 1992;107:374)
Micro: mature adipose tissue, ganglion cells and nerve fascicles; rarely cartilaginous elements
Micro images: figure 1: MRI; 2-4: lipoma (arrows to ganglion cells)
DD: lipochoristoma (also has thick walled blood vessels, smooth muscle and skeletal muscle), lipoblastic meningioma (larger, extends beyond internal auditory canal)
References: Laryngoscope 1998;108:1459
Meningioma of middle/inner ear or temporal bone
Benign tumor arising from arachnoid cells
Must exclude secondary extension from intracranial tumor
13-18% of intracranial tumors
Usually women, age 40+ years; rarely children
Internal auditory canal meningiomas are rare (AJNR Am J Neuroradiol 2006;27:2204), may mimic vestibular schwannoma (AJNR Am J Neuroradiol 2002;23:1493)
Temporal en plaque meningioma involving the middle ear or mastoid may mimic otitis media (Otol Neurotol 2006;27:992)
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: internal auditory canal (Arq Neuropsiquiatr 2003;61:659), temporal bone secretory meningioma (Pathol Res Pract 2006;202:481), within bony labyrinth (AJNR Am J Neuroradiol 2003;24:1642), cutaneous meningioma of external auditory canal (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
Micro images: meningothelial cells with psammoma bodies; meningioma infiltrating into bone (figure 3); sheets and whorls of meningothelial cells (figure 1E); various images-unknown sites; meningotheliomatous tumor with whorled syncytial pattern; left: whorled and syncytial cells with delicate chromatin; right: psammoma bodies; left: whorls of meningothelial cells with intranuclear cytoplasmic inclusions; right: paraganglioma-like growth; left: weak and focal EMA+; right: CK7+
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
First described in 1976 (Laryngoscope 1976;86:1123, Clin Otolaryngol Allied Sci 1976;1:17)
Neuroendocrine and epithelial differentiation; also called carcinoid tumor or amphicrine tumor (Ultrastruct Pathol 2001;25:73)
Rosai believes they form a continuum with carcinoid tumor and could be considered adenocarcinoid tumors
No gender preference, usually 20’s to 40’s, but wide age range
Affects all sites in middle ear
Occasionally perforates tympanic membrane and extends into external auditory canal
Not associated with chronic otitis media or cholesteatoma
Excellent prognosis; rarely is locally aggressive, invades vital structures, has regional metastases (Laryngoscope 2005;115:1660) or causes death
Case reports: 2 cases #1 (AJCP 1987;87:592), #2 (J Clin Path 1991;44:652), pleomorphic adenoma of middle ear
Treatment: complete surgical excision; mastoidectomy may be necessary for large lesions; recurs with inadequate excision
Gross: gray-white to red-brown, firm/rubbery masses; relatively well circumscribed; not encapsulated; no hemorrhage, mean 0.8 cm
Micro: variable patterns (sheets, solid, trabecular, cystic, cribriform, glandular, NOT papillary) of glands or tubules composed of uniform 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; may produce PAS+ mucin; sparse fibrous or myxoid stroma; no/rare mitotic figures, no necrosis
May have neuroendocrine differentiation morphologically and immunohistochemically
Micro images: (1) trabecular growth pattern, salt and pepper chromatin; (2) upper left: solid growth pattern with plasmacytoid cells; upper right: trabecular growth pattern with artifactual clefting; lower left: infiltrative pattern; lower right: organoid pattern; (3) tumor with infiltrative (upper right), organoid (upper central) and solid / trabecular / glandular patterns; (4) glandular pattern; (5) pagetoid tumor spread; (6) carcinoid-type pattern; (7) inner flattened cells within gland; inset shows CK7+ inner epithelial layer; (8) Figure 1-foci of mucinous differentiation; 2-CAM5.2+; 3-synaptophysin+; (9) prominent glandular pattern; (10) trabeculae and ribbons
Positive stains: keratin (90%), CK7 (90%), chromogranin (88%), CAM5.2 (81%), mucin (intraluminal), lysozyme, neuron specific enolase (50%), synaptophysin (31%), serotonin (25%), S100 (15%), CK20 (6%, focal)
Negative stains: actin
EM: desmosomes and microvilli; often membrane bound dense core granules; may have glandular differentiation (AJCP 1985;84:541)
EM images: neurosecretory granules
DD: jugulotympanic paraganglioma (zellballen surrounded by S100+ sustentacular cells), 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: Archives 2006;130:1067, Mod Path 2002;15:543 (adenoma vs. carcinoid tumor)
Papilloma of middle/inner ear or temporal bone
Rare
May be disseminated locally by surgical manipulation (Laryngoscope 1984;94:1568)
Case reports: middle ear squamous papilloma (Rev Bras Otorrinolaringol (Engl Ed) 2005;71:396), inverted papilloma of middle ear #1 (Otol Neurotol 2002;23:555), #2 with malignant transformation (J Laryngol Otol 2006;120:597), inverted papilloma of temporal bone (Laryngoscope 2002;112:140)
Gross images: papilloma of tympanic membrane and ear canal
Micro: papilloma covered by squamous or columnar cells; either exophytic (everted), inverted or oncocytic
Micro images: squamous papilloma without atypia
inverted papillomas at other sites - frontal sinus #1; #2; #3; unknown site #1; #2
Middle ear, inner ear and temporal bone tumors-malignant
Adenocarcinoma of middle / inner ear
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, doesn’t metastasize but may exhibit intracranial extension (J Neurosurg 1999;90:555)
Treatment: complete excision
Micro: resembles adenoma but with increased pleomorphism and mitotic figures and extensive infiltration of surrounding structures
DD: metastases (need good clinical history to rule out)
References: Arch Otolaryngol Head Neck Surg 1987;113:822
Aggressive papillary tumor of temporal bone / middle ear
Also called papillary adenoma of endolymphatic sac / temporal bone, adenocarcinoma of temporal bone / mastoid, endolymphatic sac tumor, low grade adenocarcinoma of probable endolymphatic sac origin, 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); also somatic mutations of VHL gene in non VHL patients (Cancer Res 2000;60:5963)
Most arise within intraosseous portion of the endolymphatic duct/sac, with precursor lesions present in VHL patients (Cancer Res 2005;65:10847)
Median age 30’s, range of 11-71 years
Symptoms: early sensorineural hearing loss, tinnitus and episodic vertigo
Xray: 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
Tumor grows slowly with only one reported metastasis (J Neurosurg Spine 2005;3:68), but may recur with inadequate excision; is infiltrative, destructive and may cause death; bleeds profusely at surgery
Case reports: women with tumor and von Hippel Lindau disease (Archives 2003;127:1387, J Clin Path 1994;47:959, Mod Path 2001;14:727), with somatic VHL mutation (Hum Path 2001;32:1272); aspirated cyst fluid analysis (Mod Path 2001;14:920); 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; often granulation tissue reaction with small vascular spaces and mixed inflammatory infiltrate is 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; resembles choroid plexus papilloma
Cytology: rare epithelial cell clusters, some with papillary features; also foamy macrophages; epithelial cells have eosinophilic and focally vacuolated cytoplasm, some with pigmented granules resembling hemosiderin, well defined cell borders, bland nuclei
Micro images: various images #1; #2; A: tumor composed of papillae lined by bland cuboidal cells extending into bone; B: tumor cells have moderate cytoplasm, round/oval bland nuclei, finely granular chromatin, indistinct nucleoli; C: EMA+; D: CK7+; papillary tumors #1; #2; papilla with hemorrhagic material; papilla with vacuolated cells and colloid-like material; papillary cystic structures (figure 2); glands contain eosinophilic colloid; papillary structures composed of cuboidal cells; PAS+ globules; A: CK7+; B: CK8+; C: CK19+; vascular endothelial growth factor+; focal area of high Ki-67+
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/CD57
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, “zellballen”, keratin negative)
References: AJSP 1988;12:790, Cancer1989;64:2292
Chondrosarcoma of temporal bone
Rare (ORL J Otorhinolaryngol Relat Spec 1998;60:58); most commonly affects petrous apex and posteromedial aspect of temporal bone
Patients may have long survival
Case reports: mesenchymal chondrosarcoma (Auris Nasus Larynx 2002;29:371), low grade myxoid chondrosarcoma (Am J Otol 1994;15:419)
References: Cancer 1986;58:2689
Giant cell tumor of temporal bone / ear
Rare
Case reports: temporal bone tumor #1 (Archives 2003;127:1217), #2 (BMC Ear Nose Throat Disord 2005;5:8), #3 (J Craniofac Surg 2006;17:797)
Micro images: various images; giant cell tumor #1; #2 (figure D-E)
References: Lin Chuang Er Bi Yan Hou Ke Za Zhi 2003;17:596
Langerhans cell histiocytosis of ear
Clonal proliferation of Langerhans cells, either isolated or part of systemic process
Usually males, teens to twenties
Common sites are middle ear and temporal bone
Rarely involves labyrinth in adults (Otol Neurotol 2004;25:27)
Xray: single or multiple sharply circumscribed osteolytic lesions
Case reports: bilateral temporal bone involvement (Pol Merkur Lekarski 2003;14:250)
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
Micro images - other sites - H&E; CD1a; various images #1; #2; #3
Cytologic images: temporal bone; spine - B: Diff Quik; C: Pap (arrow at eosinophil); D: CD1a; E: H&E;
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-)
Lymphoepithelioma-like carcinoma of middle ear
Micro: abundant lymphoid stroma
Micro images: upper - trabecular pattern of malignant cells with uniform, pale, vesicular nuclei and prominent nucleoli; lymphoid infiltrate present; bottom - EBER+; #2 (similar)
Positive stains: EBER
References: J Clin Path 1998;51:602
Malignant peripheral nerve sheath tumor of middle/inner ear
Case reports: originating in recurrent schwannoma/acoustic neuroma (J Clin Path 2004;57:109), originating in parapharyngeal space and extending into middle ear cavity (AJNR Am J Neuroradiol 2001;22:748)
Micro images: anaplastic cells; benign area within same mass
site unknown - whorling spindle cells with perivascular accentuation
Metastases to middle / inner ear
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
Clinical clues are rapid sensorineural hearing loss and progressive facial nerve weakness (Acta Otorhinolaryngol Ital 2004;24:78)
Case reports: bladder to middle ear (Ann Otolaryngol Chir Cervicofac 2002;119:349), breast infiltrating ductal carcinoma to internal auditory canal and cerebellopontine angle (Acta Otolaryngol 2005;125:1004), cardiac myxoma to temporal bone (AJCP 1989;91:221), colon adenocarcinoma to external ear canal (Ear Nose Throat J 2005;84:36), lung squamous cell carcinoma to internal auditory canal (Am J Otolaryngol 2006;27:214), prostate to internal auditory canal (Clin Neurol Neurosurg 2003;105:180)
Often associated with Paget’s disease of bone, fibrous dysplasia or radiation therapy (Am J Otol. 1997;18:230)
Aggressive, often metastasizes to lung or brain
5 year survival <15%
Case reports: metastases from tibial tumor to temporal bone in patient with Paget’s disease (J Otolaryngol 1992;21:112)
Micro images: site unknown - low power; high power
Rhabdomyosarcoma of middle / inner ear
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
Case reports: with EM (J Clin Path 1965;18:63)
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
Micro images: striated tumor cells
not necessarily from ear – A: embryonal type with small nuclear size and pleomorphism; B: alveolar type with larger nuclei and more uniform appearance
EM: actomyosin filaments, ribonucleoprotein particles, no virus-like particles
EM images: thick and thin filaments with interconnecting cross-bridging; numerous actomyosin filaments, ribonucleoprotein and glycogen particles #1; #2
DD: inflammatory polyp / granulation tissue
Squamous cell carcinoma of middle / inner ear
Rare; usually age 50’s and 60’s
Usually >20 year history of chronic otitis media or radiation therapy for intracranial tumor or middle ear inflammation
25% have associated cholesteatomas
Symptoms: long standing chronic otitis media with sudden onset of pain, otorrhea, hemorrhage
5 year survival: 39%; 10 year survival: 21%; usually no metastatic disease
Case reports: invasion into membranous labyrinth (Ann Otol Rhinol Laryngol 1983;92:290)
Treatment: radical surgery with radiation therapy, chemotherapy for advanced disease
Gross: tumor fills middle ear spaces, may invade walls of mastoid air cells, bone adjacent to carotid canal, internal auditory meatus, Eustachian tube and 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
References: Ann Otol Rhinol Laryngol 1985;94:273
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 is present
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