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HEAD AND NECK PATHOLOGY HUSNI MAQBOUL,M.D

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Page 1: 01 Oral Cavity.pdf

HEAD AND NECK PATHOLOGY

HUSNI MAQBOUL,M.D

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HEAD AND NECK PATHOLOGY

• Diseases of the following anatomic sites :

– Oral Cavity

– Upper Airways

– Ears

– Neck

– Salivary Glands

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Oral Cavity Congenital Abnormalities

• Dermoid Cysts

– Usually present at birth, may become

evident later when secondarily infected

– Seen in the midline of the floor of the

mouth

– Lined by squamous epithelium and

contain skin adnexae

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Oral Cavity Congenital Abnormalities

• Heterotopic Gastric or Intestinal

Epithelium

– Tongue and floor of the mouth

– May result in cyst formations

• Odontogenic Origin Cysts

– Newborns and older infants

– Alveolar and Palatal Mucosa

– ! No need for biopsy

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Oral Cavity Congenital Abnormalities

• Heterotopic Nerve Tissue

– Palate and Parapharyngeal Space

– Glial elemetns and ependymal clefts

– May give rise to tumors

• White Sponge Nevus

– Autosomal dominant

– Large white plaques in oral mucosa

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White Sponge Nevus

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Oral Cavity Congenital Abnormalities

• Fordyces Disease

– Presence of normal sebaceous glands

within the oral cavity

– Common occurrence

• Lingual Thyroid

• Epithelial Nests ( Organ of Chievitz)

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Oral Cavity Inflammatory Diseases

• Defense Mechanisms of the oral

mucosa

– Competitive suppression by low virulence

organisms

– Secretory IgA

– Saliva

– Dilution and irrigation by foods and

drinks

– Rich blood supply

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Oral Cavity Inflammatory Diseases

• Herpes Simplex

– Mostly caused by type I, usually trivial

sores

– Rarely Acute Herpetic Gingivostomatitis

– Small, rapidly eroded vesicles to large

bullae

– Spontaneously clear within 3 - 4 wks

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Oral Cavity Inflammatory Diseases

• Herpes Simplex

– Virus becomes dormant in local ganglia (

trigiminal) that can be reactivated, usually on

lips,around nasal orifices, and buccal mucosa

• Morphology

– Intra and intercellular edema

– Intranuclear viral inclusions ( Tzank Test )

– Multinucleated giant cells

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Oral Cavity Inflammatory Diseases

• Oral Candidiasis ( Thrush )

– Normal inhabitant of the mouth

– Causes disease in diabetic, neutropenic,

and in immunocompromised patients

– Also in xerostomia, and broad spectrum

antibiotic therapy.

– Superficial gray whitish membrane,

readily scraped of revealing erythematous

inflammatory base.

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Oral Cavity Inflammatory Diseases

• Aphthous Ulcers ( Canker Sores )

– Extremely common, painful superficial

ulcerations

– More common in the first two decades life

– Tend to be prevalent within certain

families

– Unknown cause, more painful than serious

• Behcet’ s Disease

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Oral Cavity Inflammatory Diseases

• Aphthous Ulcers ( Canker Sores )

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Oral Cavity Inflammatory Diseases

• Glossitis

– Beefy-red tongue encountered in Vit B

deficiency states, atrophy of papillae and

thinned mucosa

• Plummer -Vinson Syndrome

– Fe Deficiency, glossitis, esophageal

dysphagia

• Other causes include ill-fitting dentures, syphilis,

burns, corrosives

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Oral Cavity Inflammatory Diseases

• Geographic Tongue ( Benign Migratory Glosssitis )

– Loss of filiform papillae, with erythematous

flat zones , associated with fissuring

– Usually asymptomatic, mostly adults

– Pathology : Psoriasiform process with reactive

inflammation

– Cause unknown

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Oral Cavity Inflammatory Diseases

• Geographic Tongue ( Benign Migratory Glosssitis )

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Oral Cavity Inflammatory Diseases

• Xerostomia ( Dry Mouth )

– Dry mucosa with atrophy of the papillae

and ulceration

– Radiation therapy, drugs

– Sjogrens syndrome with inflammatory

enlargement of salivary glands.

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Oral Cavity Specific Inflammatory Diseases

• Tuberculosis

– Rare

– Painful ulcers of tongue or buccal mucosa

• Syphilis

– Gumma of tongue or palate

– Painless indurated mass

• Histoplasmosis

– Indurated ulcers, nodular lesions, verrucous

masses

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Oral Cavity

• Histoplasmosis

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Oral Cavity Specific Inflammatory Diseases

• Crohn’s Disease

– 6% of patients, sometimes as the initial

manifestation

– Lips, gingiva, vestibular sulci and buccal mucosa

– Edema, ulcers, or papulous hyperplastic mucosa

• Sarcoidosis

– Gingiva, tongue, hard palate and buccal mucosa

– Random lower lip biopsy

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Oral Cavity Specific Inflammatory Diseases

• Melkersson - Rosenthal Syndrome

– Orofacial swelling

– Plicated tongue

– Facial nerve paralysis

– Cheilitis Granulomatosa

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Oral Cavity Reactive lesions

• Non-neoplastic conditions presenting

as tumor masses

• Varying proportions of hyperplastic

epithelium, fibrous tissue, and

inflammatory cells

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Oral Cavity Reactive lesions

• Irritation Fibroma

– Buccal mucosa along the bite line

• Pyogenic Granuloma

– Highly vascular pedunculated lesion of gingiva

in children, young adults, and pregnant women

• Peripheral Giant Cell Granuloma

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Oral Cavity Reactive lesions

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Oral Cavity Reactive lesions

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Oral Cavity

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Oral Cavity Other Non-neoplastic lesions

• Extravasation Mucocele

– Stromal reaction to spillage of mucus from

traumatically injured minor salivary gland

– Mostly in lower lip

• Ranula

– Anatomic variant, blue domed cyst

sublingually

• Retention Mucocele

– Older patients, so specific site

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Oral Cavity Other Non-neoplastic lesions

• Necrotizing Sialometaplasia

– Minor, less commonly major salivary glands

– Ulcerating lesion of hard palate

– Partial necrosis of salivary gland with

regeneration and squamous metaplasia

– ? Ischemic, ? Vasculitis

– Can be pathologically confused with

malignancy

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Oral Cavity Manifestations of Systemic Diseases

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Oral Cavity Manifestations of Systemic Diseases

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Oral Cavity Manifestations of Systemic Diseases

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Oral Cavity Tumors of the Surface Epithelium

• Leukoplakia

– Clinical term denoting whitish plaque > 5mm,

that cannot be removed by rubbing and not

classified as another disease entity

– Keratosis + Dysplasia

– Oral Intraepithelial Neoplasia ( OIN )

– Some times accompanied with “Lichenoid”

histology ( Lichenoid Dysplasia )

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Oral Cavity Tumors of the Surface Epithelium

• Leukoplakia

– Buccal gingival gutter and floor of the mouth

– 2 - 5% SCC within 10 years

– Ominous features are speckled, warty or

verrucous

– Increased expression of Proliferating Cell

Nuclear Antigen

– Must be considered precancerous until proved

otherwise

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Oral Cavity Leukoplakia

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Oral Cavity, Tumors

• Erythroplasia

– Circumscribed area that may or not be

elevated with poor defined irregular margins

– Red to velvety, often granular

– Micro

• Epithelial dysplasia

• 50% transformation rate to malignancy

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Oral Cavity, Tumors

• Squamous Cell Carcinoma In situ

– The most severe degree of OIN spectrum

– 90% have red , velvety ( Erythroplastic )

component

– Induration almost always associated with

invasion

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Oral Cavity SCC In situ

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Oral Cavity Papillomatous Lesions

• Human Papilloma Virus

• Benign types 2,4,6,11,13 and 32

– Focal Epithelial Hyperplasia (Heck’s Disease)

– Verruca Vulgaris

– Condyloma Accuminatum

– Squamous Papilloma

• Malignant types 16 and 18

– Verrucous and Squamous Cell Carcinomas

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Oral Cavity Squamous Papilloma

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Oral Cavity Papillomatous Lesions

• Hairy Leukoplakia

– Seen in HIV patients

– White confluent fluffy patches along lateral

edges of the tongue

– Caused by EBV, sometimes with superimposed

candidiasis

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Oral Cavity Hairy Leukoplakia

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Oral Cavity, Tumors

• Squamous Cell Carcinoma

• General Features

– 90% of Ca of oral cavity

– Mostly between 50 and 70 years

– ! About 50% are diagnosed late and prove to

be fatal

– Tobacco, alcohol, SPHL, oral sepsis, iron

deficiency , candidiasis, HPV

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Oral Cavity, Tumors

• Squamous Cell Carcinoma

– Location

• Vermilion border of the lip

• Floor of mouth

• Lateral borders of the mobile

tongue

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Oral Cavity, Tumors

• Squamous Cell Carcinoma

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Oral Cavity, Tumors

• Squamous Cell Carcinoma

– Spread and Metastasis

• Lip : skin, orbicular muscle,buccal mucosa,

mandible, and mental nerve

• Floor : Sublingual gland, muscle, gingiva

• Tongue : Tends to remain localized longer,

eventually involves floor of the mouth and root of

the tongue

• To Cervical and Retropharyngeal lymph nodes

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Oral Cavity, Tumors

• Verrucous Carcinoma ( Ackerman’s Tumor)

• Variant of well differentiated SCC

• Most common sites are buccal mucosa and lower

gingiva

• Large, fungating soft papillary growth

• Can invade mandible, maxilla and perineural

spaces

• Exceedingly rare L/N mts. No distant mts

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Oral Cavity Tumors

• Other Microscopic Types

• Adenoid Squamous Cell Carcinoma

• Adenosquamous Carcinoma

• Basaloid Squmous Cell Carcinoma

• Spindle Cell ( Sarcomatoid Carcinoma )

• Small Cell Carcinoma

• Lymphoepithelioma - Like Carcinoma

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ORAL PATHOLOGY II

HUSNI MAQBOUL, M.D

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Minor Salivary Glands

Lower Lip Biopsy can be useful in :

– Sarcoidosis

– Cystic Fibrosis

– Sjogren’s Syndrome

– GVHD

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Tumors of Minor Salivary Glands

Salivary Gland Choristoma

Gingival nodule made up of disorganized

sero-mucinus salivary gland tissue mixed

with sebaceous glands

Adenomatoid hyperplasia

Hard palate, occasionally retomolar area

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Tumors of Minor Salivary Glands

Benign Mixed Tumor ( Pleomorphic

Adenoma )

– Makes up only half of salivary gland

tumors of the palate

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Tumors of Minor Salivary Glands

Malignant intraoral salivary gland tumors:

Adenoid Cystic Carcinoma

Prognosis is better in the palate than in parotid

or submaxillary glands

Mucoepidermoid Carcinoma

Polymorphous low-grade adenocarcinoma

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Tumors of Minor Salivary Glands

Tumors occurring predominantly in minor

salivary glands of the oral cavity:

Basal Cell ( Canalicular ) Adenoma

Predilection for upper lip

Characterized by canalicular pattern of

growth

Benign behavior

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Tumors of Minor Salivary Glands

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Tumors of Melanocytes

Ephilis and Lentigo ( Melanotic Macules )

Can present as solitary lesions, usually

lower lip of females

Multiple pigmented macules can be seen in

Peutz-Jeghers Syndrome

Melanocytic Nevi

Lips, rarely inside the oral cavity

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Tumors of Melanocytes

Malignant Melanoma

Common in people of Japanese and Black

African origin

Both pigmented and amelanotic varieties

occur

Common lymph node and distant

metastases

Extremely poor prognosis

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Tumors and tumor like conditions of

lymphoid Tissue

Malignant Lymphoma

Mostly in palatine and lingual tonsils

Can also develop in gingival areas, buccal

mucosa, or palate

Soft bulky mass ulcerated, or covered by

normal mucosa

Most cases are B-cell NHL

In 40% of cases, there is evidence of disease

outside the oral cavity

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Malignant Lymphoma

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MAJOR & MINOR

SALIVARY GLANDS

Husni Maqboul, M.D

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Swellings in the Neck

Embryonic remnants

– Thyroglossal

– Branchial cyst

Salivary gland lesions

– Cysts

– Lymphoid infiltrates

– Tumors

Thyroid gland lesions

Related to mandible

Related to carotid bifurcation

Related to cervical lymph nodes

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Normal Histology

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Normal Histology

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Sialosis

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Oncocytosis

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SIALOLITHIASIS

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SIALOADENITIS

Acute :

– Can be localized to one salivary gland, usually

parotid or submaxillary

– Manifestation of systemic viral infection ,

mumps (paramyxovirus ), EBV, coxackievirus,

influenza and parainfluenza viruses

– Acute suppurative - S.aureus, strep. and Gr-ve

Predisposing factors : Dehydration,

malnutrition, sialolithiasis, and

immunosuppression

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SIALOADENITIS

Chronic :

– Lymphocytic infiltrate that can unaccompanied

by clinical symptoms

– Various degrees of atrophy, focal obstruction

and fibrosis

– Immune mediated , more in females ,

associated with RA

– Kuttner’s tumor : Unilateral chronic sclerosing

sialoadenitis of submandibular gland.

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SIALOADENITIS

Granulomatous

– TB, sarcoidosis, duct obstruction, or malignant

tumor

– Xanthogranulomatous variant

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SIALOADENITIS

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LYMPHOEPITHELIAL CYSTS

Benign lymphoepithelial cysts

– Proliferation of branchial pouch-derived

or analogous epithelium induced by

lymphoid hyperplasia

– Present as cystic structures in upper

cervical lymph nodes or parotid

– ? Similar in origin to branchial cleft cysts

of head and neck, cysts in Hashimoto

thyroiditis, and thymic cysts

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LYMPHOEPITHELIAL CYSTS

– Warthin tumor is considered by some as the

oncocytic variant of benign

lymphoepithelial cysts

– HIV patients have similar lesions plus solid

lymphoepithelial lesions

Pathology

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LYMPHOEPITHELIAL

CYST

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Mikulicz’s Syndrome

Diffuse and bilateral

enlargment of salivary and

lacrimal glands

Sjogren’s Syndrome

Xerostomia

Keratoconjunctivitis

Rheumatoid Arthritis

Hypergammaglobulin

Malignant

Lymphoma

TB Sarcoidosis

Mikulicz’s Disease

Benign lymphoepithelial

lesion

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Lymphoid Infiltrates

Mikulicz’s Syndrome: Diffuse and bilateral

enlargement of salivary and lcarimal glands

– Lymphoma , sarcoidosis, TB, and

Mikulicz’s Disease ( Benign Lymphoepithelial

Lesion ) is the most common cause of

Mikulicz’ Syndrome.

– Striking bilateral and symmetric enlargement of

salivary glands

– Systemic autoimmune disorder with clonal

expansion of B-lymphocytes

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Lymphoid Infiltrates

Mikulicz’s Disease ( Benign Lymphoepithelial

Lesion )

– Can evolve into full-blown lymphoma

– Pathology : Lymphoid infiltrate with reactive

geminal centers and solid nests of epimyoepithelial

islands representing collapsed acini

– Similar changes of minor salivary glands of oral

cavity with scant or absent myoepithelial islands

– Systemic manifestations ~ Sjogren’s Syndrome

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Mikulicz’s Disease ( Benign

Lymphoepithelial Lesion )

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Other Non-neoplastic Lesions

Lymphoid Disorders : Reactive or

inflammatory changes of intraparotid

lymph node can be confused with primary

salivary gland tumors

Keratinous Cysts of epidermal type can

involve mostly the parotid gland

Amyloidosis : Part of a generalized

process or as a localized pseudotumoral

mass

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Epithelial Tumors Tumors with Stromal Differentiation

Benign Mixed Tumor ( Pleomorphic Adenoma )

– Most common neoplasm of salivary glands

– Most frequent in women in fourth decade

– Most commonly in the parotid where it arises in the

tail (50%) or anterior portion (25% ) of the superficial

lobe

– The remaining 25% arise in the deep lobe and present

as pharyngeal mass

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Epithelial Tumors Tumors with Stromal Differentiation

Benign Mixed Tumor ( Pleomorphic Adenoma )

– Rubbery mass with bosselated surface that may grow

to a large size

– Though well circumscribed, small extensions can be

seen protruding into surrounding normal tissue

– Biphasic appearance due to intimate admixture of

epithelium and stroma

– Epithelial glandular component with squamous foci

– Stroma : Fibromyxoid with chondroid islands

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Pleomorphic Adenoma

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Epithelial Tumors Tumors with Stromal Differentiation

Malignant Mixed Tumor

Malignant transformation of a pre-existing tumor

– 5% - 10% of neoplasms

– Malignancy limited to epithelial component

– Mts to L/N, lung, bone, and abdominal organs

“True”malignant mixed tumor without pr-existing

benign tumor

– Biphasic malignancy ( Ductal carcinoma and

Chondrosarcoma )

– Highly aggressive and rapidly lethal

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Malignant Mixed Tumor

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Epithelial Tumors Tumors with oxyphilic “oncocytic” change

Warthin’s Tumor ( Cystadenoma

Lymphomatosum Papilliferum )

– Almost exclusively in the parotid gland

– More common in males with statistical

relationship with smoking

– Can be multicentric and is bilateral in 10%

– Lobulated mass with multicystic appearance

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Warthin’s Tumor

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Epithelial Tumors Monomorphic Neoplasms

Basal Cell Adenoma

– Adult patient with slight female predeliction

– Mostly in parotid gland

– Encapsulated, often cystic

– Tubular, trabecular or solid pattern of growth

– Canalicular adenoma variant

– Can be associated with dermal cylindromas

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Basal Cell Adenoma

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Epithelial Tumors Monomorphic Neoplasms

Basal Cell Adenocarcinoma

– Malignant counterpart of Basal Cell Adenoma

– Infiltrative quality with cytologic atypia,

perineural spread, and vascular permeation

– Parotid gland is the predominant site, and the

peak incidence is in the sixth decade

– Local recurrence and MTS to L/N and lungs

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Myoepithelioma

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Epithelial Tumors Tumors with Clear Cell Change

Do not constitute A homogenous or specific

type, clear cell change can be seen in :

– Myoepithelioma

– Sebaceous neoplasms

– Mucoepidermoid carcinoma

– Acinic cell carcinoma

– Metastatic renal cell carcinoma

Most of these tumors occur in the oral cavity

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Epithelial Tumors Mucoepidermoid Carcinoma

Most cases are located in parotid gland

Most common malignant salivary gland tumor

in children

Microscopic types : Mucinous ( mostly well

differentiated) , Squamous, Intermediat and

Clear

Low grade have 5 y survival of 98%

Hige grade have 5 y survival of 56% , associated

with local recurrence and L/N mets

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Epithelial Tumors Mucoepidermoid Carcinoma

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Epithelial Tumors Adenoid Cystic Carcinoma

Slow growing, but highly malignant neoplasm

with remarkable capacity for recurrence

Most common malignant tumor of minor

salivary glands

In the parotid, it is less common than

mucoepidermoid and acinic cell carcinomas

Solid appearance and infiltrative pattern of

growth

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Epithelial Tumors Adenoid Cystic Carcinoma

Micro : Cribriform pattern of growth ,

pseudocysts, small true glandular spaces and

characterestic is invasion of perineurial spaces

– Tubular pattern recurrence rate 59%, 15 y surv .39%

– Classic cribriform recurrence rate 89%15y surv. 26%

– Solid pattern recurrence rate 100% 15 y surv. 5%

Mts to lungs, infrequently to L/N

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Epithelial Tumors Adenoid Cystic Carcinoma

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Small Cell Carcinoma

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Malignant Lymphoma

Can arise from an intraparotid lymph node ( with

features of nodal lymphoma ), or in the gland itself

The large majority involve parotid gland

Most present as unilateral mass

Nearly all are of B-cell origin

Can arise on the backgorund of Mikulicz’s

disease, small cleaved ( MALT )

Very rare H.D and plasmacytoma

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Other Primary Neoplasms

Vascular Tumors

– Hemangioma is the most common salivary gland tumor

in children

Lipoma d.d sialosis ( lipomatosis )

Schwannoma

Embryoma ( Sialoblastoma )

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General Features of Salivary

Gland Tumors

Relative incidence and malignancy

– Twelve times more frequent in the parotid than in

the submaxillary

– Majority are benign, mostly benign mixed tumor

– Parotid gland - Incidence of malignancy was 17%

most common mucoepidermoid

– Submaxillary and palatal - incidence of malignancy

was 38% - 44% , mostly adenoid cystic

– Most tumors are unilateral and single, bilaterality

and multiplicity seen in Warthin tumor

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General Features of Salivary

Gland Tumors

Diagnosis

– Excisional biopsy

– Incisional biopsy

– True cut biopsy and F.N.A cytology

– Frozen section

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General Features of Salivary

Gland Tumors

Prognosis : Influenced by clinical staging,

location, and microscopic type

– Malignant tumors of submaxilary gland have

higher incidence of recurrence and metastases

than parotid tumors of the same type

– Adenoid cystic carcinoma has better prognosis

in the palate, intermediate in parotid, and worst

in submaxillary

– Presence of facial nerve palsy is an ominous

prognostic sign

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General Features of Salivary

Gland Tumors