HEAD AND NECK PATHOLOGY

HUSNI MAQBOUL,M.D

HEAD AND NECK PATHOLOGY

Diseases of the following anatomic sites : Oral Cavity Upper Airways Ears Neck Salivary Glands

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

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

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

White Sponge Nevus

Oral Cavity
Congenital Abnormalities Fordyces Disease
Presence of normal sebaceous glands within the oral cavity Common occurrence

Lingual Thyroid Epithelial Nests ( Organ of Chievitz)

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

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

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

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.

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

Oral Cavity
Inflammatory Diseases
Aphthous Ulcers ( Canker Sores )

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

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

Oral Cavity
Inflammatory Diseases
Geographic Tongue ( Benign Migratory Glosssitis )

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.

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

Oral Cavity
Histoplasmosis

Oral Cavity
Specific Inflammatory Diseases
Crohns 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

Oral Cavity
Specific Inflammatory Diseases
Melkersson - Rosenthal Syndrome Orofacial swelling Plicated tongue Facial nerve paralysis Cheilitis Granulomatosa

Oral Cavity
Reactive lesions Non-neoplastic conditions presenting as tumor masses Varying proportions of hyperplastic epithelium, fibrous tissue, and inflammatory cells

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

Oral Cavity
Reactive lesions

Oral Cavity
Reactive lesions

Oral Cavity

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

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

Oral Cavity
Manifestations of Systemic Diseases

Oral Cavity
Manifestations of Systemic Diseases

Oral Cavity
Manifestations of Systemic Diseases

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 )

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

Oral Cavity
Leukoplakia

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

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

Oral Cavity
SCC In situ

Oral Cavity
Papillomatous Lesions
Human Papilloma Virus
Benign types 2,4,6,11,13 and 32 Focal Epithelial Hyperplasia (Hecks Disease) Verruca Vulgaris Condyloma Accuminatum Squamous Papilloma Malignant types 16 and 18 Verrucous and Squamous Cell Carcinomas

Oral Cavity
Squamous Papilloma

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

Oral Cavity
Hairy Leukoplakia

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

Oral Cavity, Tumors

Squamous Cell Carcinoma

Location
Vermilion border of the lip Floor of mouth Lateral borders of the mobile tongue

Oral Cavity, Tumors

Squamous Cell Carcinoma

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

Oral Cavity, Tumors

Verrucous Carcinoma ( Ackermans 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

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

ORAL PATHOLOGY II

HUSNI MAQBOUL, M.D

Minor Salivary Glands

Lower Lip Biopsy can be useful in :

Sarcoidosis Cystic Fibrosis Sjogrens Syndrome GVHD

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

Tumors of Minor Salivary Glands

Benign Mixed Tumor ( Pleomorphic Adenoma ) Makes up only half of salivary gland tumors of the palate

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

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

Tumors of Minor Salivary Glands

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

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

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

Malignant Lymphoma

2/9/2013

MAJOR & MINOR SALIVARY GLANDS

Husni Maqboul, M.D

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

Normal Histology

2/9/2013

Normal Histology

2/9/2013

Sialosis

2/9/2013

Oncocytosis

2/9/2013

SIALOLITHIASIS

2/9/2013

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 2/9/2013 immunosuppression

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 Kuttners tumor : Unilateral chronic sclerosing sialoadenitis of submandibular gland.

2/9/2013

SIALOADENITIS

Granulomatous
TB, sarcoidosis, duct obstruction, or malignant tumor Xanthogranulomatous variant

2/9/2013

SIALOADENITIS

2/9/2013

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 2/9/2013

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

2/9/2013

LYMPHOEPITHELIAL CYST

2/9/2013

Malignant Lymphoma

TB

Sarcoidosis

Mikuliczs Syndrome
Diffuse and bilateral enlargment of salivary and lacrimal glands Mikuliczs Disease
Benign lymphoepithelial

Xerostomia Keratoconjunctivitis

Rheumatoid Arthritis
Hypergammaglobulin

lesion

Sjogrens Syndrome

Lymphoid Infiltrates

Mikuliczs Syndrome: Diffuse and bilateral enlargement of salivary and lcarimal glands
Lymphoma , sarcoidosis, TB, and

Mikuliczs 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 2/9/2013 expansion of B-lymphocytes

Lymphoid Infiltrates

Mikuliczs 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 ~ Sjogrens Syndrome

2/9/2013

Mikuliczs Disease ( Benign Lymphoepithelial Lesion )

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

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

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

Pleomorphic Adenoma

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

Truemalignant mixed tumor without pr-existing benign tumor

Biphasic malignancy ( Ductal carcinoma and Chondrosarcoma ) Highly aggressive and rapidly lethal

Malignant Mixed Tumor

Epithelial Tumors
Tumors with oxyphilic oncocytic change

Warthins 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

Warthins Tumor

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

Basal Cell Adenoma

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

Myoepithelioma

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

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

Epithelial Tumors
Mucoepidermoid Carcinoma

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

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

Epithelial Tumors
Adenoid Cystic Carcinoma

Small Cell Carcinoma

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 Mikuliczs disease, small cleaved ( MALT ) Very rare H.D and plasmacytoma

Other Primary Neoplasms

Vascular Tumors
Hemangioma is the most common salivary gland tumor in children

Lipoma d.d sialosis ( lipomatosis ) Schwannoma Embryoma ( Sialoblastoma )

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

General Features of Salivary Gland Tumors

Diagnosis Excisional biopsy Incisional biopsy True cut biopsy and F.N.A cytology Frozen section

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

General Features of Salivary Gland Tumors