Salivary Gland Diseases

Salivary Gland

Diseases

Cells in Salivary Glands and Ducts

Acinus Intercalated

Striated

Excretory

Myoepithelial cell

•1- Functional Disorders:1(Xerostomia

•2- Developmental1(Aplasia2(Atrasia

3(Aberrancy•3- Infectious (sialadenitis):

.ABacterial:1(Acute bacterial sialadenitis.

2(Chronic sialadenitis..BViral:

•Mumps•

Classification Of SG Diseases

4- Cysts:1. Mucous retention cyst

2. Mucous extravasation cyst

3. Ranula5- Obstructive

• Sialolithiasis

6- Autoimmune

• Sjogren`s Syndrome

7- Tumor like lesions

• Sialoadenosis

Classification of SG Diseases

7-Neoplastic1)Benign

a) Pleomorphic Adenoma

b) Monomorphic andenoma

I. Whartin’s tumor

II. Oncyocytoma

2)Malignant

a) Malignant Pleomorphic Adenoma AndCarcinoma Ex Pleomorphic Adenoma

b) Adenocystic Carcinoma

c) Mucoepidermoid Carcinoma

•Xerostomia

Functional Disorders

•Definition:

➢Means dry mouth

➢It may be temporary or chronic (persistent)

➢Temporary xerostomia is of no clinical significance

➢Persistent xerostomia is of grave consequences

•Classification:

➢Primary, due to defective glands.

➢Secondary, due to defects outside the glands

•Normal Salivary Flow:

–1 – 2 ml/minute , 1 – 2 Liters/Day

Xerostomia

•Causes:

1.Developmental (Salivary gland aplasia)

2.Loss of body fluids as:

➢Impaired fluid intake

➢Hemorrhage

➢Vomiting and diarrhea

3.Medications as antihistamines, diruetics and antidepressants

4.Radiation therapy to the head and neck

5.Systemic diseases as Sjogren’s syndrome and diabetes

6.Local Factors as smoking and mouth breathing

Xerostomia

•Oral Manifestations:

1.Sever dental caries (especially cervical caries).

2.Sever periodontal diseases

3.Atrophied and ulcerated oral mucosa and tongue

4.Superimposed infection, particularly candida albicnans

fungus due to changes in oral flora because of the reduction

in the cleansing and antimicrobial activity of the saliva

5.Difficulty in speech, mastication and swallowing

6.Loss of taste

7.Inability to wear dentures

Xerostomia

1.Remove the cause if possible

2.Check for any associated drug contributing to xerostomia

3.Frequent small sips of water

4.Prescribe saliva substitutes (artificial saliva)

5.Suggest sugar-free gum

6.Maintain good oral hygiene

7.Chlorohexidine (0.2%) rinses

8.Control caries with fluoride application

9.Monitor for candidosis (antifungal drugs)

10.Treat difficulties with dentures

11.Observe regularly for possible ascending parotitis or chronic sialoadenitis

Xerostomia - Management

Infections

•Definition:

•Acute suppurative inflammation of the parotid

•Cause and pathogenesis:

–Predisposing factors are :

1.xerostomia resulting from postoperative dehydration (One

of the common causes of acute bacterial sialadenitisis is

Recent surgery because the patient has been kept without

food or fluids and has received medications that produce

xerostomia)

2.Irradiation

3.Sjogren’s syndrome

Acute suppurative parotitis(Acute Bacterial Sialadenitis), (Acute ascending

parotitis)

–The causative organisms :

–Staphylococci, streptococci and pneumococci

▪The mode of infection is ascending i.e. via the duct and

rarely blood born.

▪The disease is now very rare due to the invention of

antibiotics and proper post-operative care

parotitis)

•Clinically:

✓Mainly affect the parotid gland and may be bilateral .

✓Painful swelling of the gland with uplifting of the ear lobe

✓The overlying skin is red, shiny and tense

✓In severe cases edema and difficulty in opening the mouth

(trismus)

✓Purulent discharge or pus from the affected duct

✓Fever and malaise

parotitis)

Histopathologic features

✓ Accumulation of neutrophils is observed within the ductal

system and acini.

Treatment

The treatment of acute sialadenitis includes:

✓ Appropriate antibiotic therapy

✓ Rehydration of the patient to stimulate salivary flow.

✓ Surgical drainage may be needed if there is abscess

formation.

parotitis)

•Definition:•This is a chronic inflammation of the salivary glands

•Cause and pathogenesis:

–Usually results form recurrent or persistent ductal obstruction caused by :

1.Sialolithiasis

2.Congenital strictures

3.compression by an adjacent tumor

Chronic Sialadenitis

•Clinically:

✓Painless or painful swelling of the affected gland which become worse at mealtime when salivary flow is stimulated.

✓Chronic sialadenitis can affect the parotid gland, submandibular gland, or the minor salivary glands.

•Histologically:

✓Atrophy of the acini

✓Ductal hyperplasia and dilatation.

✓Lymphocytic infiltration.

✓Fibrosis of the stroma

•Treatment:

–If possible remove the obstruction

–More often the gland has to be excised and

biopsied to exclude the possibility of neoplasms

Sialadenitis

Nonspecific chronic

sialadenitis reveals

dilatation of ducts,

interstitial infiltration of

lymphocytes and

fibrosis

Sialadenitis

Marked interstitial

fibrosis, infiltration

of chronic

inflammatory cells

and acinar atrophy

Mumps (Epidemic Parotitis)

▪ Definition:

Acute viral infection of the parotid

▪ Cause and pathogenesis:

• The causative virus is paramyxovirus which is RNA.

• The mode of infection is droplet infection.

• The virus reaches the gland through the duct or more commonly via blood.

• One attack produces permanent immunity.

• The incubation period is 2 - 3 weeks.

• The virus could be detected in saliva and blood (viraemia) prior to the appearance of the disease by 2 - 3 days using complement fixing antibodies

▪ Clinically:

• Fever and malaise.

• Enlargement of one parotid followed by enlargement of the other one within 2 - 3 days - rarely enlargement of both parotids occurs

• The enlargement is firm - painful - elevate the lobule of the ear and causing trismus

• No pus formation unless secondary infection occurs

• The disease is self limiting and spontaneous regression occurs after 2 - 3 weeks

▪ Histologically:

• Degeneration of the acini.

• Acute inflammatory cell infiltration

▪ Complications (rare, usually in adults):

• Extension to other salivary glands.

• Hepatitis.

• Pancreatitis

• C.N.S. affection

• Orchitis and ovaritis which may lead to sterility

▪ Prevention:

• Vaccination with MMR

▪ Treatment:

• Supportive treatment (analgesics & antipyretics)

• Plenty of fluids and soft diet

• Maintain good oral hygiene

Obstructive disorders

•Definition:•calcified structures that develop within the

•salivary ductal system

•Clinically:➢Occurs usually in adults.

➢Usually in submandibular more than parotid because the duct of the submandibular is more tortuous and the secretion is more viscous. The direction of the saliva flow is antigravity.

➢The stone occurs in the duct or in the salivary gland.

➢The condition is rare in edentulous mouths.

SialolithiasisSialolith (Salivary Calculus)

•Pathogenesis:➢They arise from deposition of calcium salts

•around a nidus of:

.athickened mucus

.bMicroorganisms

.cdesquamated ductal epithelial cells

.dforeign body (that enter the ducts of a salivary gland from the mouth)

➢Calculus consists mainly of calcium carbonate and phosphate in addition to some trace minerals.

•Radiographic picture:•They could be detected in X-ray as a radiopaque mass.

•Complication:•The stone causes obstruction and secondary infection of the

involved gland. It may cause mucous retention cyst.

•Features:➢Tender enlargement of the gland and the duct become visible in

the floor of the mouth.

➢Periodic swelling and pain occur within the affected gland. Swelling usually develop at meal time.

➢If the stone is located toward the terminal portion of the duct, a hardmass may be palpated beneath the mucosa that have a yellowish color.

Sialolithiasis – Salivary Stones

•Wharton's duct is much more commonly

involved by a sialolith than is

Stensen's duct. In many cases, the

stone is difficult to see and often no

intraoral manifestations are

evident

•Radiographs are helpful in detecting the stone, or stones, as in this good example.

Sialolithiasis – Salivary

Stones

Treatment•Small sialoliths of the major glands

sometimes can be treated

conservatively by gentle massage of the

gland in an effort to bring the stone

toward the duct orifice.

•Larger sialoliths usually need to be

removed surgically.

•If significant inflammatory damage

has occurred within the gland, and in

case of minor gland sialoliths, the gland

may need to be removed.

•The usual result of duct

obstruction is

inflammation of the

gland. The changes

consist of ductal

dilatation, acinar

degeneration, chronic

inflammation and fibrosis

•This higher power

displays the remnants

of degenerating

mucous acini (green

arrow) surrounded by

lymphocytes and

plasma cells with

areas of early fibrosis

(yellow arrow).

Cysts of Salivary Glands

1. Mucous Retention Cyst

2. Mucous Extravasation Cyst

3. Ranula

Mucocele

Cysts of the salivary

glandsI- Mucous retention cyst

• Definition:

A cyst affecting minor salivary glands due retention of

mucous.

• Pathogenesis:

Partial obstruction of the duct by stone or stenosis

due to inflammation.

• Clinical picture:

➢Usually affects lip, tongue or buccal mucosa.

➢ small swelling which is fluctuant.

➢Pain may become worse at meal time.

• Histopathology:

➢Epithelial lining: compressed ductal epithelium

ranging from pseudo stratified to stratified squamous.

➢ connective tissue wall is minimally inflamed.

➢ lumen of the cyst contains mucin.

glandsII- Mucous extravasation cyst

• Definition:

A pseudocyst affecting minor salivary glands due

extravasation of mucous into connective tissue as a

result of torn duct.

• Pathogenesis:

Trauma.

• Clinical features:

➢Usually affects lip, tongue or buccal mucosa.

➢ small swelling which is fluctuant.

➢Pain may become worse at meal time.

• Histopathology:

An area of spilled mucin surrounded by a

granulation tissue that is infiltrated by inflammatory

cells usually includes neutrophils and macrophages.

glandsIII- Ranula

• Definition:

A mucous retention or mucous extravasation cyst

occurring in the floor of the mouth related to

submandibular salivary gland.

• Pathogenesis:

Partial obstruction or Trauma.

glandsIII- Ranula

• Clinically:

A large fluctuant bluish swelling in the floor of the

mouth.

• Histopathology:

Mucous retention or mucous extravasation cyst.

glandsIII- Ranula

Mucous Retention Versus

Mucous Extravasation

Ranula

•Ranula (salivary extravasation phenomenon;

mucous retention phenomenon;

mucocele): A large compressible bluish

soft tissue enlargement of the floor of the mouth

Ranula

•Large mucocele of

the left sublingual gland with extension across the

midline.

Autoimmune diseases

•Definition:

–An autoimmune disease that principally involves salivary and

lacrimal glands resulting in xerostomia (dry mouth) and

xerophthalmia (dry eyes).

Sjogren’s Syndrome

Sjogren’s

Syndrome

•Types:

1.Primary Sjogren`s syndrome,

–(Sicca Syndrome, Benign Lymphoepithelial Lesion),

characterized by:

•Dryness of the mouth “Xerostomia”

•Dryness of the eyes “Xerophthalmia”

1.Secondary Sjogren`s Syndrome

–characterized by:

•Dryness of the mouth

•Dryness of the eyes

•Other systemic diseases such as Rheumatoid arithritis

and lupus erythematosis.

•Etiology:

1.Autoimmune

2.Retroviral infection, suspected but not

proven yet

•Clinically:

–Age: over 40 years.

–Sex: usually females (9– 1).

–Features:

1.Enlargement of lacrimal glands with keratoconguctivitis

sicca: Dryness, redness, burning sensation of eyes and

sensation of presence of a foreign body in the eye.

•Clinically:

2.Oral manifestations:

.aXerostomia.

•(Difficulty in swallowing, Altered taste, The tongue often becomes

fissured and exhibits atrophy of the papillae, The oral mucosa is dry

and may be red and tender (usually as a result of candidiasis),

Difficulty in wearing dentures Increased risk for dental caries

(especially cervical caries).

.bEnlargement of salivary glands (particularly parotid): diffuse,

firm, bilateral. It may be non painful or slightly tender

.cAny other autoimmune disease e. g. rheumatoid arthritis or lupus

erythematosus.

•Sjogren's syndrome: The

patient has diffuse firm nontender

enlargement of the left parotid gland,

accompanied by xerostomia and xerophthalmia

Sjogren’s Syndorme – Consequences of

Xerostomia

Atrophy of tongue papillae

(bald tongue) and angular

chelitis with inflammation of

the oral mucous membrane

resulting from Xerostomia

•Histopathology:

1.Atrophy of the acini.

2.Ductal hyperplasia forming masses of epithelial and myoepithelial

cells called epimyoepithelial islands

3.Dense lymphocytic infiltration with or without germinal centers,

thus the disease is described as a lymphoepithelial lesion.

4.85 % of minor salivary gland show the same picture particularly

those of the lip.

5.There is a great risk of Malignant transformationto lymphoma

(more common in primary Sjogren)

•Differential Diagnosis:

1(Malignant lymphoma:

–there is:

1.Absence of epimyoepithelial islands

2.Atypical lymphocytes

3.Invasion of the connective tissue septa and the capsule

2(Papillary cystadenoma lymphomatosum

•Serologic Abnormalities:

1.Increased sedimentation rate

2.Numerous auto-antibodies may be found,

including

(arheumatoid factor.

(banti-Sjogren's syndrome-A (SS-A).

(canti-Sjogren's syndrome-B (SS-B).

✓Antibodies SS-A and SS-B may be seen in

association with both primary and

secondary Sjogren's syndrome.

•Sialography:

–The injected material will escape

from the ducts and produce

radiopaque mass scattered within

the gland due to significant ductal

and acinar damage

–This picture is called sialoectasis

or snow storm appearance or

branchless fruit laden tree

Sjogren’s Syndorme – Sialography

This picture is

called sialoectasis

or snow storm

appearance or

branchless fruit

laden tree

•Diagnosis:

1.Labial gland biopsy.

2.Sialography.

3.Serological tests.

•Treatment:

▪No available treatment

▪However management of xerostomia and xerophthalmia is essential (Best managed symptomatically, Artificial saliva and artificial tears and good oral hygiene.

▪Treatment with immunosuppressive drugs e.g. corticosteroids is contraindicated

•Complications:

▪Malignant lymphoma in 6% of patients.

▪Squamous cell carcinoma is rare.

–For these reasons treatment with

immunosuppressive drugs e.g. corticosteroids

is contraindicated

Sialoadenosis

Definition

Sialoadenosis is a non-neoplastic, non-inflammatory

enlargement of salivary glands, most usually affecting the

parotids bilaterally.

Sialoadenosis

▪ Etiology

• Alcoholism

• Diabetes mellitus

• Drugs (sympathomimetics)

• Bulimia and obesity

• Endocrine disturbances

• Idiopathic

▪ Histologically

• Hypertrophy of serous acini

• Edema of the interstitial connective tissue

Sialoadenosis

Enlarged parotid (P) and

submandibular (SM)

glands. The patient has

alcoholic cirrhosis and

diabetes, which results in

sialosis, a benign

inflammatory enlargement.

This condition usually does

not require surgical

management. Note the

prominent masseter muscle

which can be confused

with glandular

enlargement.

Lymphocytic infiltrate destroying salivary gland in Sjogren’s

syndrome.

Lymphocytic infiltrate destroying salivary gland in Sjogren’s

syndrome.

Sjogren’s

Syndrome

•Introduction••Both major or minor glands are affected by salivary gland

tumors.

••Most cases are found within the parotid. Approximately 80% of parotid tumors are benign. Approximately 60% of submandibular tumors are benign. The sublingual gland, are uncommon site for neoplasms, the majority are malignant.

••The palate is the most common site for minor salivary gland neoplasms.

•In minor salivary glands, however, the benign‐malignant ratio is closer to 1:1.

SG Tumors

Histogenesis of SG tumors

.IEpithelial tumors

.ABenign (Adenomas)

1.Pleomorphic adenoma.

2.Monomorphic adenoma:

•-Papillary Cystadenoma Lymphomatosum (Warthin'stumor).

•-Oncocytoma.

.AMalignant (Carcinomas)

1.Malignant pleomorphic adenoma.

2.Mucoepidermoid carcinoma.

3.Adenoid cystic carcinoma.

Classification of SG Tumors

.IINon-epithelial tumors

••Lymphomas arising in patients with Sjogren’s

syndrome or within parotid lymph nodes.

••Sarcomas and benign mesenchymal tumors

arising from stroma of salivary gland (fibroblasts,

blood vessels, fat cells and nerves).

••Metastases to the salivary gland.

Classification of SG Tumors

.1Slow growth.

.2Long duration.

.3No pain.

.4No facial nerve palsy.

.5Movable (except palate because of the tight palatal mucosa).

.6Unattached to skin or mucosa (except palate).

.7No ulceration of skin or mucosa unless by trauma

Features Suggestive of Benignancy

1.Rapid growth; rapid spurt.

2.Short duration.

3.Pain, often severe.

4.Facial nerve palsy

5.Induration.

6.Fixed to overlying skin or mucosa.

7.Ulceration of skin or mucosa

Features Suggestive of Malignancy

Facial Paralysis

Ulcerated mass

Benign Salivary Gland Tumors

•Definition:

✓Benign tumor of salivary gland characterized by variable histologic appearances.

✓The term pleomorphic or mixed denotes a diverse histological appearance.

✓The term mixed is not accurate as it is an epithelial neoplasm and not a mixed one. The myoepithelial cells is responsible for the mesenchyme-like components of the tumor.

Pleomorphic Adenoma

(Mixed Tumor of The Parotid)

Pleomorphic

Adenoma

•Clinically:

➢Frequency: the most common SG neoplasm.

➢Age: middle age (40-50).

➢Site: major salivary gland more than minor.

•Among major salivary gland parotid is mostly affected.

•Among minor glands, palatal glands are mostly affected.

➢Features: swelling - painless - slowly growing – not fixed to the surrounding structures - does not ulcerate unless by trauma - does not affect facial nerve if it occurs in the parotid.

➢If neglected, the tumor can reach a huge size.

Pleomorphic Adenoma

(Mixed Tumor Of The Parotid)

•Histologically:

1.Connective tissue capsule of varying thickness and completeness some tumor cells lie inside and outside this capsule this fact is responsible for the high recurrence rate of this tumor.

2.The tumor is composed of a mixture of ductal epithelium and myoepithelial cells within a mesenchyme-like background.

3.The epithelium form duct structures or may be found in nests or sheets. Ducts are lined by inner row of cuboidal cells, and outer layer of Clear myoepithelialcells and contain eosinophilic material produced by myoepithelial cells.

Pleomorphic Adenoma

•Histologically:

4.Myoepithelial cells may appear angular or spindle with deeply stained

nucei. It may appear as plasmacytoid cells (resemble plasma cells,

rounded with eccentric nucleus and eosinophilic cytoplasm).

5.Rarely, squamous metaplasia

•with keratin production may be seen

Pleomorphic Adenoma

•Histologically:

5.Mesenchyme-like stroma The highly

characteristic stromal changes are

believed to be produced by

myoepithelial cells.

•a. Chondroid appearance: The clear

myoepithelial cells may become rounded,

swollen and progressively separated by

accumulated mucoid basophilic material

so they become similar to cartilage cells.

Pleomorphic Adenoma

–b. Myxoid appearance: Due to mucoid

accumulation between stellate

myoepithelial cells with long

anastomosing processes.

–c. Fibroid appearance: Due to presence

of spindle shaped myoepithelial cells.

–d. Osseous tissue:On rare occasions, bone

may be found due to stromal metaplasia.

Pleomorphic Adenoma

•Histogenesis:

➢It was thought that the tumor is mixed this theory was an

attempt to explain the unusual matrix of this tumor.

➢Now it is believed that the tumor is not mixed. It arises

from the epithelial and myoepithelial cells of intercalated

ducts.

Pleomorphic Adenoma

•Behavior and complications:

➢Benign but there is high recurrence rate

–with multifocal appearance of the recurrent tumor.

➢Rare cases may progress into malignant pleomorphic

adenoma.

➢Proximity to facial nerve in parotid tumor may cause nerve

damaged at operation and leads to facial paralysis (The

facial nerve passes through the parotid gland)

–Treatment

–Wide surgical excision.

Pleomorphic Adenoma

•This is an example of what can happen if a mixed tumor should recur. Although this is

a bizarre example, it demonstrates well the

multinodularity so typical of recurrent tumor. The reason for this is that during the first

surgical procedure many small pieces of tumor were distributed throughout the

operative site and each formed the nidus for a

recurrent mass.

Pleomorphic Adenoma – With Malignant

Transformation

Pleomorphic Adenoma

•Pleomorphic adenoma: A firm well

circumscribed soft tissue enlargement fills

up the palatal vault. The lesion has a blue

color but it does not blanch upon pressure.

This means it is not vascular

Pleomorphic Adenoma

Pleomor

Adenom

•Definition:

–A benign tumor of salivary gland consists of two

elements, epithelial and lymphoid and occurs almost

exclusively in the parotid gland.

Warthin’s Tumor

(Papillary Cystadenoma Lymphomatosum)

•Clinically:

–Age: Usually old ages (above forty years).

–Sex: males more than femals(associated with

–cigarette smoking).

–Site: Parotid gland. it is bilateral in most cases.

–Features:

–Small slowly growing nodule occupying

–a superficial position just beneath the capsule

–of the gland.

–The lesion feels soft and fluctuant to palpation.

Warthin’s Tumor

•Histopathologic Features:

•(2 elements): Epithelial element and

Lymphoid element

•1.This tumor is capsulated and consists of

multiple cystic spaces contains mucoid

material and surrounded by lymphoid

stroma rich in lymphocytes and show

germinal centers.

Warthin’s Tumor

•Histologically: (2 elements):

–2.cystic spaces are lined by double layers of ductal epithelial cells

with rounded nucleus and finely granular eosinophilic cytoplasm

(similar to oncocytic cells , the granules represent mitochondria).

–3. The inner luminal layer consists of Tall columnar cells with

centrally placed, palisaded, hyperchromatic nuclei.

Warthin’s Tumor

•Histologically: (2 elements):

–4. The cells of the outer layer are Small

cuboidal or polygonal with vesicular

nuclei and separated from the stroma by

basement membrane.

–5. The lining epithelium demonstrates

multiple papillary folding or projections

into the cystic spaces.

Warthin’s Tumor

Two layers of pink staining epithelium

Warthin’s Tumor

Two layers of pink staining epithelium

Warthin’s Tumor

Cystic spaces lined

by double layer of

epithelial cells

resting on a dense

lymphoid stroma

sometimes bearing

germinal centers

Warthin’s Tumor

Double layer of

epithelial cells:

Surface epithelial

cells are columnar

cells having abundant,

finely granular,

eosinophilic

cytoplasms, imparting

an oncocytic

appearance which rest

on layers of cuboidal

to polygonal cells

–Histogenesis:

–It arises from entrapment of salivary gland tissue within

parotid lymph nodes during development.(due to late

encapsulation of parotid gland, some small lymph nodes are

enclosed within the parotid gland).

–Treatment:

–Surgical excision.

Warthin’s Tumor

•Special features of Warthin’s tumor:

▪Occurs only in the parotid gland

▪The second most common benign tumor of parotid gland

▪May be unilateral or bilateral

▪May occur as multiple lesions

▪More common in males (26:1)

▪May be associated with acute onset of pain and a sudden increase in size (papillary cystadenoma lymphomatosumsyndrome)

▪Ear symptoms (tinnitus, deafness, earache).

Warthin’s Tumor

Malignant Tumors of Salivary Glands

•2 types exist:

1.Primary malignant pleomorphic adenoma:

–Which is malignant from the start, the malignant cells are the

epithelial and myoepithelial cells, hence metastasis in the remote

area consists of both types of cells.

2.Malignant pleomorphic adenoma EX benign

pleomorphic adenoma:

–It is a salivary gland neoplasm characterized by malignant

transformation of the epithelial component of a previously

benign pleomorphic adenoma. It is aggressive and considered to

be a high grade malignancy.

Malignant Pleomorphic Adenoma

•Clinical Features:

•It occurs in old age (60 years).

•painful, rapidly growing mass.

•Facial nerve paralysis (if the neoplasm affects the

parotid).

•Ulceration and fixation.

Carcinoma Ex Pleomorphic Adenoma

•Histopathologic Features:

➢Areas of typical benign pleomorphic adenoma usually can be

found within the tumor.

➢Areas of malignant epithelial component ,characterized by

cellular pleomorphism and abnormal mitotic activity.

•Treatment:

•Radical surgical removal.

Carcinoma Ex Pleomorphic Adenoma

•Definition:

•A malignant tumor of salivary gland consists of mucous,

epidermoid and intermediate cells

•It is the most common malignancy in children.

Mucoepidermoid Carcinoma

–Clinical features:

▪Occurs most commonly in the parotid gland

▪The palate is the most common intraoral site.

▪It affects a wide age range (10-70 years)

▪Two grades have been described.

–The low grade malignancy type which resembles benign

tumors. The high grade type which appears as a swelling -

painful - rapidly growing - fixed to the surrounding tissues -

cause facial nerve paralysis if the tumor occurs in the parotid.

Tumors of intermediate behavior have been also describe

–Clinical Features

–Neoplasms of minor salivary glands appears as:

–•Swelling or ulcerated mass.

–•Swelling may be fluctuant and have a blue color (resemble

mucocele).

–•It is painless in early stages.

–•May affects the palate, buccal mucosa,

–Tongue or lip.

–Histogenesis:–Arises from epithelial cells of excretory ducts

–Histopathologic Features:

•Mucoepidermoid carcinoma is composed of a mixture of:

••Mucous–producing cells.

••Squamous (epidermoid) cells.

••Intermediate cell , which is believed

•to be a progenitor of both the mucous

•and the epidermoid cells.

••The mucous –secreting cells are

•cubical or columnar with foamy

•cytoplasm. They tend to line the cystic

•spaces, or arranged in solid sheets .

••The epidermoid cells form solid sheets

•or line the cystic spaces. These cells

•are of squamous epithelial type

•having a polygonal shape .

••Intermediate cells are smaller than

•Either mucous or epidermoid cells .

•They appearas small basaloid cells.

•1. Low-grade tumors show

••high proportion of mucous cells,

••prominent cyst formation,

••minimal cellular atypia.

•2. High-grade tumors show

••high proportion of solid sheets of

•Squamous and intermediate cells

••cells demonstrate considerable

•pleomorphism, and mitotic activity.

••few cystic spaces.

•3. Intermediate-grade tumors :

•show features between those of the low-grade and high-grade

neoplasms.

Mucoepidermoid carcinoma. Cystic spaces lined by mucous secreting cells (M) and intermediate cells (I).

Mucoepidermoid carcinoma, mandible. Cystic spaces (C) and nests of neoplastic squamous (N) cells along with bone spicules at the periphery (B).

•High

Behavior of Mucoepidermoid Carcinoma

Intermediate

Mucous Cells Epidermoid CellsMucous &

Intermediate

•Central Mucoepidermoid Carcinoma:

•A rare situation in which mucoepidermoid carcinoma might occur centrally inside the bone. The lesion may arise from:

1.Entrapment of retromolar mucous glands within the mandible.

2.Inclusion of the submandibular gland within the mandible (latent bone cyst).

3.Neoplastic transformation of mucous secreting cells (goblet cells) present in the wall of dentigerous cyst.

4.Neoplastic transformation of mucous secreting cells (goblet cells) present in the lining of the maxillary sinus.

–Treatment:–Surgical excision.

•Like all malignancy of

salivary glands,

mucoepidermoid

carcinoma tends to

ulcerate with metastasis

to regional lymph nodes

•Definition:▪A malignant tumor of salivary

–gland characterized by formation of

–multiple cysts.

▪It is a high grade salivary gland malignancy.

▪It is the most common minor salivary gland malignancy.

▪Because of its distinctive histopathologic features, it was

originally called a "cylindroma”.

▪The use of the term cylindroma should be avoided because it

does not denote the malignant nature of the tumor and because

the same term is used for a benign skin tumor.

Adenoid Cystic Carcinoma

Cylinderoma

•Clinically:

•Frequency: rare

•Age: middle age

•Site: minor more than major among the minor salivarygland palatal glands are mostly affected. Among the major salivary gland parotid is mostly affected.

•Features: ......................................+

•Tendency of the tumor to spread through perineurallymphatics thus early pain and tenderness is a constant feature of this tumor + late metastasis via blood.

Cylinderoma

•Histopathologic Features

.aThe adenoid cystic carcinoma is composed of a mixture of myoepithelial cells and ductal cells.

.bThe ductal cells are small and cuboidal, with deeply stained basophilic nuclei and little cytoplasm.

.cMyoepithelial cells have clear cytoplasm and darkly staining angular nuclei.

•Three histopathologic patterns are recognized:(1) cribriform, (2) tubular, and (3) solid.

Cylinderoma

•(1) Cribriform pattern

••is the most common pattern.

••It is characterized by islands of basaloid

•epithelial cells that contain multiple cystic spaces

resembling swiss cheese.

••These spaces often contain basophilic mucoid material.

Adenoid Cystic Carcinoma – Cylinderoma

•(2) tubular pattern

••The tumor cells occur as multiple small ducts or tubules.

within a hyalinized stroma.

••The duct like spaces are lined by an inner row of cuboidal

epithelial cells and an outer zone of myoepithelial cells.

•(3) solid pattern

••It consists of large islands or sheets of cuboidal epithelial

cells.

••This type may also show more pleomorphism and mitotic

activity than the other forms.

••This type is the most aggressive type.

Cribriform pattern

Tubular pattern

Solid pattern

•Behavior:

–The cribriform and the tubular patterns are of low grade

malignancy, while the solid pattern is considered to be grade

malignancy.

•Histogenesis:

–Adenoid Cystic Carcinoma originate from intercalated duct

cells and myoepithelial cells

–Treatment

–Radical surgical removal.

cribriform

pattern

–Most

common

–“swiss

cheese”

appearance

cribriform pattern

–Most common

–“swiss cheese”

appearance

tubular pattern–

Layered cells

forming duct-like

structures–

Basophilic

mucinous

substance

solid pattern–Solid

nests of cells

without cystic or

tubular spaces

This is the typical

cribriform pattern of tumor

cells in adenoid cystic

carcinoma. There are small

spaces lined by ductal cells

and larger spaces

containing basement

membrane-like material.

The pattern overall

resembles a slice of Swiss

cheese

At Hi Mag, the duct

lining cells are visible

in small clusters with

pink cytoplasm. The

stellate cells

surrounding the ducts

and the larger round

spaces are

myoepithelial variants

Low power of

adenoid cystic

carcinoma

showing

cribriform

pattern at the left

and tubular

pattern at the

Perineural & Endoneural Invasion

•What is the significance of this section? How does it

influence your differential diagnosis?

•This section demonstrates perineural and endoneural

invasion. Given this finding, you can safely eliminate

pleomorphic adenoma from the differential diagnosis.

Remember

•What is the significance of this section? How does it influence your differential diagnosis?

•Bilateral parotid swelling may occur in:

••Mumps

••Acute bacterial sialadenitis

••Sjogren’s syndrome

••Warthin’s tumor

1.Necrotizing Sialometaplasia

2.Sialoadenosis

Tumor-Like Lesions

•Definition

–This is a tumor-like lesion usually affects minor

salivary glands of the palate.

•Etiology

–Exact etiology is unknown; however the

presumed cause is ischemia of the minor

salivary glands resulting from infection, trauma,

irradiation, or irritation caused by ill-fitting

dentures.

Necrotizing Sialometaplasia

•Clinically

–Resembles carcinoma clinically and histologically and is readily mistaken for it.

–Most commonly develops in males in the form of a painless deep, sudden ulcer of the hard palate usually

opposite to the first molar.

–Lesions resolve without treatment within 6 to 10 weeks.

•Histologically

–Biopsy is usually necessary to rule out squamous cell carcinoma or a minor salivary gland malignancy. Review

of the tissue by a pathologist well versed in head and neck pathology is essential.

–Characteristic histologic findings include necrosis of the acini, ductal squamous metaplasia, and chronic

inflammatory cell infiltration.

•Necrotizing sialometaplasia

at the typical site. The

ulceration is full-thickness, deep

and there has been

involvement and exposure of the underlying bone

•Painful ulceration of the palate of 2 weeks duration

•This lesion is more superficial.

The clinical similarity to

squamous cell carcinoma is

obvious. Note, however, the surrounding

inflammatory erythema

•The sub-acute variant,

unusually occurring

bilaterally (note erythema also).

Biopsy confirmed necrotizing

sialometaplasia, but the lesions

did not ulcerate.

•Ulcerated lesion with squamous proliferation at edge

of ulcer

•Note extent and

worrying complex

architecture of

squamous proliferatio

•Definition

–Sialoadenosis is a non-neoplastic, non-

inflammatory enlargement of salivary glands,

most usually affecting the parotids bilaterally.

Sialoadenosis

•Etiology

–•Alcoholism

–•Diabetes mellitus

–•Drugs (sympathomimetics)

–•Bulimia and obesity

–•Endocrine disturbances

–•Idiopathic

•Histologically

–•Hypertrophy of serous acini

–•Edema of the interstitial connective tissue

Sialoadenosis

•Enlarged parotid (P) and submandibular

(SM) glands. The patient has alcoholic

cirrhosis and diabetes, which results in

sialosis, a benign inflammatory

enlargement. This condition usually does

not require surgical management. Note the

prominent masseter muscle which can be

confused with glandular enlargement.

Sialoadenosis

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