Salivary Gland Pathology

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Salivary Gland Pathology
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Mucocele

• Mucus Extravasation Phenomenon Mucus Escape
  Reaction

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Mucocele Clinical Features

• Mucoceles typically appear as bluish, dome-shaped
  swellings if superficial or of normal color if
  deep in the tissues.
• They are typically covered by an intact
  epithelium.
• They may be slightly painful due to an
  accompanying acute inflammatory reaction

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Mucocele Clinical Features

• Mucoceles are most common on the lower lip
  (approx. 81 ), floor of the mouth, ventral
  tongue and buccal mucosa (in order of decreasing
  frequency)
• They rarely occur in the upper lip
• Mucoceles are more common in children and
  adolescents
• They are a common oral lesion

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Location of Mucoceles

• Location Number of Cases of All Cases
• Lower lip 1477 81.0
• Floor of mouth 106 5.8
• Ventral tongue 106 5.8
• Buccal mucosa 87 4.8
• Palate 26 1.4
• Retromolar 10 0.5
• Unknown 12 0.7
• Upper lip 0 0.0
• Total 1824 100.0
• From Neville, et al., Third Edition

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Mucocele Cause and Treatment

• They are typically caused by traumatic severance
  of the salivary gland excretory duct.
• Occasionally, mucoceles will rupture
  spontaneously and heal without treatment.
• Surgical excision, if needed, is the treatment of
  choice. Removal of the adjacent minor salivary
  gland helps prevent recurrence.

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Mucocele VS Mucus Retention Cyst VS Salivary Duct
Cyst
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Mucocele Prognosis and Significance

• The prognosis is excellent, although occasional
  mucoceles will recur, necessitating re-excision,
  especially if the feeding glands are not removed.

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Ranula Clinical Features

• A ranula is the term used for mucoceles that
  occur in the floor of the mouth.
• The term ranula is derived from the Latin word
  for frog.
• They appear as dome-shaped, fluctuant swellings
  unless they are deep in the tissue.
• Typically, they are lateral to the midline.
• Plunging or cervical ranulas dissect through
  the myohyoid muscle to produce swelling in the
  neck.

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Ranula Cause

• The cause is usually severance of the sublingual
  gland duct although severance of the
  submandibular duct may be the cause.
• Blockage of the duct with a salivary gland stone
  (sialolith) may produce a ranula which, in this
  case, would be a true mucous cyst.

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Ranula Treatment and Significance

• Treatment may consist of marsupalization and/or
  surgical removal.
• Marsupalization is often unsuccessful
  necessitating removal.
• The unilateral, lateral location may help
  distinguish the ranula from a midline dermoid
  cyst as well as color and consistency of the
  lesion.

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Salivary Duct Cyst

• Mucus Retention Cyst
• Mucus Duct Cyst
• Sialocyst

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Salivary Duct Cyst Clinical Features

• These lesions typically appear as solitary,
  asymptomatic, mobile, non-tender swellings
  covered by an intact epithelium.
• They are usually the same color as the
  surrounding tissue.

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Salivary Duct Cyst Clinical Features

• Salivary duct cysts are more common in adults
  over the age of 50 years.
• The palate, cheek, floor of the mouth are the
  more common locations. They are uncommon in the
  upper lip and rare in the lower lip.
• They may occur in the major glands with the
  parotid being the most common major gland site.

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Salivary Duct Cyst Cause, Treatment and
Significance

• Salivary duct cysts are caused by blockage of the
  salivary gland excretory duct by a sialolith.
• They are treated by conservative surgical
  excision.
• Recurrence is not anticipated if the associated
  gland is removed.
• Rarely, patients may have multiple mucus
  retention cysts then surgical excision is
  performed only on the more problematic lesions.

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Sialolithiasis

• Salivary Calculi
• Salivary Stones

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Sialolithiasis Clinical Features

• Salivary gland stones occur most often in the
  submandibular gland ducts but they may also occur
  in the minor glands particularly of the upper lip
  and buccal mucosa.
• Young and middle-aged adults are most frequently
  affected.
• Patients frequently present with episodic pain
  and swelling particularly around mealtime.

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Sialolithiasis Clinical Features

• Stones in the terminal ducts can usually be
  palpated.
• If the sialolith is well calcified, it may appear
  on radiograph as a radipaque mass.
• Minor gland stones are often asymptomatic.

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Sialolithiasis Cause, Treatment and Significance

• Deposition of calcium salts around a nidus of
  debris in the duct lumen occurs but the exact
  cause of this is unknown.
• The blockage of the duct and resultant
  inflammation can cause significant damage to the
  gland.
• Small sialoliths can sometimes be removed by
  gentle message, sialagogues, moist heat, or
  increased fluid intake. Larger stones are
  removed surgically.
• Stones in minor glands/ducts are best treated by
  surgical removal including the associated gland.

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Sialadenitis

• Inflammation of the salivary glands can arise
  from various infectious and non-infectious
  causes.
• The most common viral infection is mumps.
• Most bacterial infections arise as a result of
  ductal obstruction or decreased salivary flow.
• One of the more common causes of sialadenitis is
  recent surgery.

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Sialadenitis Clinical Features

• Acute bacterial sialadenitis is most common in
  the parotid where it produces a painful swelling.
  The overlying skin may be erythematous and the
  patient may have low-grade fever, trismus and
  purulent discharge.
• Chronic sialadenitis is associated with periodic
  swelling and pain.
• Subacute necrotizing sialadenitis is more common
  in young (males?) adults. The lesion usually
  involves the minor glands of the hard or soft
  palate. It appears as a painful nodule, which
  does not ulcerate or slough like necrotizing
  sialometaplasia.

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Sialadenitis Cause

• The inflammation of the glands can arise for
  various causes as noted previously. While mumps
  is the most common viral cause, other viruses
  such as Coxsackie A, ECHO, choriomeningitis,
  parainfluenza and cytomegalovirus may be the
  cause.
• The most common cause of acute bacterial
  sialadenitis is Staphylococcus aureus but
  streptococci and a host of other bacteria have
  been implicated at different times.
• Medications that can induce xerostomia can
  predispose the patient to infection.
• Non-infectious causes include Sjögren syndrome,
  radiation therapy, sarcoidosis and some allergens.

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Sialadenitis Treatment, Prognosis and
Significance

• Acute sialadenitis is treated by antibiotic
  therapy and rehydration to stimulate salivary
  flow.
• Surgical drainage may be required if abscesses
  occur.
• Management of chronic sialadenitis depends upon
  the severity and duration of the condition.
• Subacute necrotizing sialadenitis is
  self-limiting and usually resolves in 2 weeks.
• Significant inflammatory destruction of the
  salivary gland can occur requiring its surgical
  removal.

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Cheilitis Glandularis Clinical Features

• This uncommon inflammatory condition
  characteristically affects the minor glands of
  the lower lip producing swelling and eversion.
• The duct openings are dilated and inflamed and
  digital pressure often produces a mucopurulent
  secretion.
• This entity is most common in middle-aged and
  older males but can affect any population.
• Superficial suppurative (Baelz disease) and deep
  suppurative (cheilitis glandularis apostematosa)
  are more progressive stages with greater
  swelling, inflammation, suppuration and
  ulceration.

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Cheilitis Glandularis Cause, Treatment and
Significance

• The cause is uncertain but it is associated with
  actinic damage, tobacco, syphilis, poor hygiene
  and heredity.
• The treatment of choice for persistent cases is
  vermilionectomy (lip shave), which usually gives
  satisfactory results.
• A significant percentage of cases (18-35 ) have
  been associated with the development of squamous
  cell carcinoma.

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Sialorrhea

• Overproduction of saliva can produce drooling and
  chocking.
• Patients with idiopathic paroxysmal sialorrhea
  may have short episodes of excessive salivation
  associated with prodromal nausea or epigastric
  pain.

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Sialorrhea Cause

• Minor sialorrhea is associated with aphthous
  ulcers and ill-fitting dentures.
• It is also associated with rabies, heavy metal
  poisoning, gastroesophageal reflux disease and
  certain drugs such as lithium and cholinergic
  agonists as well as neurologic disorders such as
  cerebral palsy.

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Sialorrhea Treatment, Prognosis and Significance

• In cases which are mild and/or transitory, no
  treatment is needed.
• In persistent severe case, therapeutic
  intervention may be required.
• Anticholinergic medications, surgical correction
  and speech therapy have all been used in
  appropriate situations.
• Social embarrassment, soiling of clothes and bed
  linens can be significant problems.

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Xerostomia Clinical Features

• Xerostomia, dry mouth, is more common in females
  and the elderly.
• With decreased salivary flow, the saliva becomes
  foamy or thick and ropey. There is a lack of
  polling of saliva in the floor or the mouth and
  the mucosa appears dry.
• The dorsal tongue is often fissured with atrophy
  of the filiform papilla.

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Xerostomia Clinical Features

• Mastication and swallowing may be difficult.
• The clinical findings do not always correspond to
  the degree of salivary flow.
• The incidence of oral candidiasis increases as
  does dental decay, especially cervical and root
  caries radiation caries (xerostomia-related
  caries).

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Xerostomia Cause, Treatment and Significance

• Xerostomia can result from a variety of causes
  including salivary gland aplasia, aging,
  smoking, mouth breathing, local radiation
  therapy, Sjögren syndrome, HIV infection and some
  medications. Causes are highlighted in a later
  slide.

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Xerostomia Cause, Treatment and Significance

• Xerostomia is difficult to treat. Treatments
  have included use of artificial salivas,
  surgarless candy and gum, sialagogues such as
  pilocarpine and changing the patients
  medication.
• Xerostomia predisposes to increased dental caries
  and oral candidiasis.

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Benign Lymphoepithelial Lesion

• Myoepithelial Sialadenitis
• Other Terms Which Were Formerly Used Mikulicz
  Disease and Mikulicz Syndrome

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Benign Lymphoepithelial Lesion Clinical
Features

• Most cases develop as a component of Sjögren
  syndrome and present as bilateral salivary gland
  swelling although other cases are unilateral.
• This entity has a predilection for adults with a
  mean age of 50 years.
• It has a female gender predilection (60-80 of
  cases).
• Eight-five percent of the cases occur in the
  parotid gland.
• It usually appears as a firm, diffuse swelling
  and the lesions may be asymptomatic or present
  with mild pain.

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Benign Lymphoepithelial Lesion Cause

• Mikulicz disease (clinical presentation of
  painless swelling of the lacrimal and salivary
  glands due to a lesion histologically diagnosed
  as benign lymphoepithelial lesion.
• Mikulicz syndrome (clinical presentation of
  lacrimal and salivary glands secondary to other
  disease entities such as TB, sarcoid and
  lymphoma.
• Many case of so-called Mikulicz disease may be
  examples of Sjögren syndrome.
• Role of autoimmunity

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Benign Lymphoepithelial Lesion Treatment,
Prognosis Significance

• Benign lymphoepithelial lesions frequently
  necessitate surgical removal of the involved
  gland.
• The prognosis in most cases is good.
• However, patients with this entity have an
  increased risk for developing a lymphoma (?40
  times?). There is also a malignant counterpart
  termed malignant lymphoepithelial lesion or
  lymphoepithelial carcinoma. This is more common
  in Inuits and Asian populations.
• Some cases originally diagnosed as benign
  lymphoepithelial lesion are actually early-stage
  MALT lymphomas, which are low-grade tumors.

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Sjögren Syndrome Clinical Features

• Sjögren syndrome is a chronic, systemic
  autoimmune disorder that principally involves the
  salivary and lacrimal glands.
• It predominantly affects middle-aged and older
  adults with 80-90 of them being women.
• The principal oral symptom is xerostomia.
• A third to a half of all patients have diffuse,
  firm enlargement of the major salivary glands,
  usually bilaterally.

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Sjögren Syndrome Clinical Features

• The swelling may be painful or just slightly
  tender and these sensations may be intermittent
  or persistent.
• The patient may have dry skin and nasal and
  vaginal dryness as well.
• Fatigue is a common symptom and many patients are
  depressed.
• Sialography often reveals punctate sialectasia
  with a fruit-laden, branchless tree pattern.

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Sjögren Syndrome Clinical Features

• Two forms of Sjögren syndrome are recognized
• Primary (sicca syndrome sicca means dry) with
  dry eyes and dry mouth and no other autoimmune
  disorder.
• Secondary where the patient manifest dry eyes and
  mouth plus another associated autoimmune disease
  such as rheumatoid arthritis, SLE, scleroderma

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Sjögren Syndrome Cause, Treatment and
Significance

• It is an autoimmune disease with increased ESR,
  elevated serum immunoglobulin levels,
  autoantibodies especially anti-SS-A and
  anti-SS-B. About 75 of patients are positive
  for rheumatoid factor.
• Treatment is mostly supportive. Artificial tears
  and saliva, sugarless gum and candy, sialagogues
  (pilocarpine) fluoride applications to prevent
  caries and antifungals if candidiasis is a
  problem.
• Sjögren syndrome patients have up to a 40 times
  increased risk for developing a lymphoma, which
  are usually a non-Hodgkin B-cell lymphoma, and
  for developing caries and oral candidiasis.

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Box 11-2 Revised International Classification
Criteria for Sjögren Syndrome

• I. Ocular symptoms A positive response to at
  least one of the following questions
• A. Have you had daily, persistent, troublesome
  dry eyes for more than 3 months?
• B. Do you have a recurrent sensation of sand or
  gravel in the eyes?
• C. Do you use tear substitutes more than three
  times a day?
• II. Oral symptoms A positive response to at
  least one of the following questions
• A. Have you had a daily feeling of dry mouth for
  more than 3 months?
• B. Have you had recurrently or persistently
  swollen salivary glands as an adult?
• C. Do you frequently drink liquids to aid in
  swallowing dry food?
• III. Ocular signs Objective evidence of ocular
  involvement defined as a positive result for at
  least one of the following two tests
• A. Schirmer I test, performed without anesthesia
  (5 mm in 5 minutes)
• B. Rose bengal score or other ocular dye score
  (4 according to van Bijsterveld's scoring
  system)
• IV. Histopathology
• In minor salivary glands (obtained through
  normal-appearing mucosa) focal lymphocytic
  sialadenitis, evaluated by an expert
  histopathologist, with a focus score 1, defined
  as a number of lymphocytic foci (which are
  adjacent to normal-appearing mucous acini and
  contain more than 50 lymphocytes) per 4 mm2 of
  glandular tissue

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Box 11-2 Revised International Classification
Criteria for Sjögren Syndrome (CONTINUED)

• V. Salivary gland involvement Objective
  evidence of salivary gland involvement defined by
  a positive result for at least one of the
  following diagnostic tests
• A. Unstimulated whole salivary flow (1.5 ml in
  15 minutes)
• B. Parotid sialography showing the presence of
  diffuse sialectasias (punctate, cavitary, or
  destructive pattern), without evidence of
  obstruction in the major ducts
• C. Salivary scintigraphy showing delayed uptake,
  reduced concentration, and/or delayed excretion
  of tracer
• VI. Autoantibodies Presence in the serum of the
  following autoantibodies
• A. Antibodies to Ro(SS-A) or La(SS-B) antigens,
  or both

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Box 11-2 Revised International Classification
Criteria for Sjögren Syndrome (CONTINUED)

• RULES FOR CLASSIFICATION
• Primary Sjögren Syndrome
• In patients without any potentially associated
  disease, primary Sjögren syndrome is defined as
  follows
• I. Presence of any four of the six items is
  indicative of primary Sjögren syndrome, as long
  as either item IV (histopathology) or VI
  (serology) is positive
• II. Presence of any three of the four objective
  criteria items (items III, IV, V, and VI)
• Secondary Sjögren Syndrome
• In patients with a potentially associated disease
  (e.g., another well-defined connective tissue
  disease), presence of item I or item II plus any
  two from among items III, IV, and V considered
  indicative of secondary Sjögren syndrome
• Exclusion Criteria
• Past head and neck radiation treatment
• Hepatitis C infection
• Acquired immunodeficiency syndrome (AIDS)
• Preexisting lymphoma
• Sarcoidosis
• Graft-versus-host disease (GVHD)

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Sialadenosis (Sialosis) Clinical Features

• Sialadenosis is a non-inflammatory disorder
  characterized by salivary gland enlargement, most
  common of the parotid.
• Most cases present as a slowly developing,
  painless swelling of the parotids.
• Most cases present with bilateral involvement.
• Decreased salivary secretion may occur.
• Sialography demonstrates a leafless tree
  pattern.

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Sialadenosis Causes

• This condition is frequently associated with an
  underlying systemic problem, which may endocrine,
  nutritional or neurogenic as seen in the
  accompanying slide.
• The best known conditions include diabetes
  mellitus, general malnutrition, alcoholism and
  bulimia.
• It is thought that these conditions cause a
  dysregulation of the autonomic innervation of the
  salivary acini producing the enlargement.

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Sialadenosis Treatment and Significance

• Clinical management is often unsatisfactory
  because it may be difficult or impossible to
  control the underlying condition.
• Mild cases present few problems and are not
  treated. If swelling presents cosmetic concerns,
  surgery may be used to correct the problem.
• Observation of the enlargement may lead to the
  diagnosis of the underlying systemic problem.

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Adenomatoid Hyperplasia of the Minor Salivary
Glands

• Uncommon lesion that is characterized by
  localized swelling that mimics a neoplasm.
• Its pathogenesis is uncertain while local trauma
  has been considered.
• The palatal minor salivary glands are most
  commonly affected.
• Most cases have occur in the fourth to sixth
  decades of life and the lesion presents as a
  sessile, painless swelling that may either soft
  or firm in consistency and is usually the color
  of the surrounding tissue.
• Biopsy is necessary to establish the diagnosis.
  Once the diagnosis is established no further
  treatment is necessary and the lesion should not
  recur.

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Necrotizing Sialometaplasia

• This lesion is an uncommon, locally destructive
  inflammatory condition of salivary glands.
• Because of its worrisome clinical presentation,
  biopsy usually is indicated to rule out the
  possibility of malignancy.

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Necrotizing Sialometaplasia Clinical Features

• The palatal salivary glands are most commonly
  affected with 2/3 of the cases being unilateral.
• It is more common in adults with a mean age of 46
  years.
• Males are more commonly affected.
• Initially the lesion presents as a non-ulcerated
  swelling often associated with pain or
  paresthesia.
• Within 2-3 weeks the tissue becomes necrotic and
  sloughs leaving a crater-like ulcer. The pain
  typically subsides when the tissue sloughs.

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Necrotizing Sialometaplasia Cause

• The cause of necrotizing sialometaplasia is
  uncertain but most authorities believe it is
  related to ischemia leading to tissue infarction.
• Predisposing factors may include traumatic
  injury, dental injections, ill-fitting dentures,
  upper respiratory tract infections, adjacent
  tumors and previous surgery.

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Necrotizing Sialometaplasia Treatment,
Prognosis Significance

• As noted previously biopsy is usually necessary
  to rule out malignancy. Once the diagnosis is
  made, no specific treatment is necessary.
• The lesion typically resolves on its own accord
  in 5-6 weeks.
• The significance of this lesion comes from the
  fact that it mimics a malignant process, both
  clinically and microscopically.

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Sites of Occurrence of Primary Epithelial
Salivary Gland Tumors
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Frequency of Malignancy for Salivary Tumors at
Different Sites
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Parotid Tumors
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Submandibular Tumors
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Minor Salivary Gland Tumors
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Location of Minor Salivary Gland Tumors
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Palatal Salivary Gland Tumors
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Location of Labial Salivary Gland Tumors
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Intraoral Minor Salivary Gland Tumors Percentage
Malignant by Site
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Salivary Gland Tumors
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Pleomorphic Adenoma (Benign Mixed Tumor)
Introduction

• This tumor is easily the most common salivary
  neoplasm.
• Pleomorphic adenomas are derived from a mixture
  of ductal and myoepithelial elements.
• This mixture gives rise to a remarkable diversity
  of microscopic appearances both among different
  pleomorphic adenomas and within any one tumor.

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Pleomorphic Adenoma (Benign Mixed Tumor)
Introduction

• Neither the term pleomorphic nor mixed are
  entirely accurate in describing this neoplasm.
• The basic pattern of the neoplasm is highly
  variable but rarely are individual cells actually
  pleomorphic.

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Pleomorphic Adenoma (Benign Mixed Tumor)
Introduction

• Although the tumor often has prominent
  mesenchymal appearing stroma, it is not truly a
  mixed neoplasm that is derived from more than one
  germ layer. These stromal changes are believed
  to be produced by the myoepithelial cells.
• Occasionally, salivary tumors are seen that are
  composed almost entirely of myoepithelial cells
  with no ductal elements. These tumors are called
  myoepitheliomas.

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Pleomorphic Adenoma Clinical Features

• As indicated, this lesion is the most common
  salivary gland neoplasm representing from 53-77
  of all parotid tumors, 44-68 of submandibular
  tumors and from 38-43 of all minor gland
  tumors.
• Benign mixed tumors are most commonly diagnosed
  between the ages of 30-50 years and there is a
  slight female gender predilection.
• They typically appear as slow growing, painless
  masses.

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Pleomorphic Adenoma Clinical Features

• In the parotid, they occur more commonly in the
  superficial lobe and initially the lesion is
  movable.
• Intraorally, they are most common in the palate
  (posterior-lateral) followed by the upper lip and
  buccal mucosa.
• The intraoral lesion is typically
  smooth-surfaced, dome-shaped and non-ulcerated
  (if traumatized the pleomorphic adenoma can be
  ulcerated).
• In the hard palate, pleomorphic adenomas will be
  non-mobile due to the mucosa being tightly bound
  to the underlying bone.

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Pleomorphic Adenoma Cause, Treatment, Prognosis
Significance

• The cause of this tumor is unknown.
• Pleomorphic adenomas are best treated by surgical
  excision.
• With adequate surgery, there is a 95 cure rate.
• With inadequate surgery, multifocal seeding
  occurs. In such cases multiple recurrences are
  not unusual.
• Malignant transformation is a potential
  complication but the rate is lt 5 of all cases.
  Transformation typically occurs many years
  (10-15) after the tumor is originally recognized.

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Oncocytoma (Oxyphilic Adenoma) Clinical Features

• The oncoytoma is more common in older adults with
  a peak prevalence in the 8th decade.
• This neoplasm has a slight female predilection
• It is most commonly seen in the major salivary
  glands where it appears as a slow-growing firm,
  painless swelling.

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Oncocytoma Cause and Treatment

• The cause of this rare neoplasm is not known.
• The treatment of choice is surgical excision and
  in the parotid gland usually consists of a
  partial parotidectomy to avoid violation of the
  tumor capsule along with preservation of the
  facial nerve if possible.

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Oncocytoma Prognosis and Significance

• The prognosis is usually good with a low rate of
  recurrence. However sinonasal oncocytomas can be
  locally aggressive and are usually managed as
  low-grade malignancies.
• Oncocytomas should not be confused with
  Oncocytosis (nodular oncocytic hyperplasia),
  which is a metaplastic transformation of ductal
  and acinar cells to oncocytes. This benign
  condition is found primarily in the parotid gland
  in older adults and requires no treatment.

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Warthin Tumor (Papillary Cystadenoma
Lymphomatosum)

• Warthin Tumor occurs as a slow-growing, painless,
  firm to fluctuant nodular mass.
• It is found almost exclusively in the parotid
  gland.
• Between 5-14 of the cases are bilateral.
• Warthin tumor is more common in older adults with
  a peak in the 6th to 8th decades.
• Early literature indicated the lesion was almost
  exclusively seen in males but recent reports
  indicated a more equal gender presentation.

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Warthin Tumor Cause, Treatment, Prognosis and
Significance

• The cause is unknown. Some authors have
  suggested it results from heterotopic salivary
  gland tissue occurring within the parotid lymph
  nodal tissue. Smokers are said to have an
  eightfold greater risk than non-smokers.
• Treatment consists of surgical removal and there
  is a 6-12 recurrence rate.
• Since some tumors are multicentric in nature, is
  it a recurrence or a proliferation of another
  nodule.
• Malignant Warthin tumors do occur but these are
  exceedingly rare.

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Canalicular Adenoma Clinical Features

• This tumor typically presents as a slow-growing,
  painless, firm to somewhat fluctuant mass, which
  may or many not be blue in color.
• It occurs most frequently in older adults (7th
  decade) and has a female gender predilection
  (1.2-1.8 1).
• The most common site for this tumor, which occurs
  almost exclusively in the minor salivary glands,
  is the upper lip (75 ) followed by the buccal
  mucosa.

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Canalicular Adenoma Cause, Treatment, Prognosis
Significance

• The cause of canalicular adenomas is unknown.
• Local surgical excision is the treatment of
  choice.
• There is a low rate of recurrence but again since
  this lesion is often multifocal in nature, the
  lesion may not be a recurrence per se.

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Basal Cell Adenoma

• This benign salivary gland tumor is so named
  because of the basaloid appearance of its tumor
  cells.
• The basal cell adenoma has a parotid site
  predilection although cases have occurred in the
  upper lip and buccal mucosa.
• It has a peak prevalence in the 7th decade and is
  more common in females.

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Basal Cell Adenoma

• Clinically, it appears as a slow-growing, freely
  movable mass similar to the pleomorphic adenoma.
• The membranous subtype appears to be hereditary
  and is often seen in conjunction with skin
  appendage tumors.
• Treatment consists of complete surgical removal
  and recurrence is rare except for the membranous
  subtype.
• There is a malignant counterpart termed a basal
  cell adenocarcinoma.

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Ductal Papillomas

• Sialadenoma Papilliferum
• Intraductal Papilloma
• Inverted Ductal Papilloma

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Ductal Papillomas Clinical Features

• All 3 lesions are uncommon to rare.
• All are more common in adults.
• Sialdenoma papilliferum is an exophytic papillary
  growth which occurs more commonly on the palate
  and has a female predilection (21).

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Ductal Papillomas Clinical Features

• Intraductal papilloma is more common on the lips
  where it appears as submucosal swelling.
• The inverted ductal papilloma is seen on the
  lip/mandibular vestibule area, where it appears
  as an asymptomatic submucosal nodule.

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Ductal Papillomas Cause, Treatment, Prognosis
Significance

• What causes the ductal papillomas is unknown.
• Treatment consists of conservative surgical
  excision in each case and recurrence is rare.
• The pathologist should be aware of these lesions
  to prevent misdiagnosis and treatment.

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Mucoepidermoid Carcinoma Clinical Features

• This is the most common malignant salivary gland
  neoplasm in the United States.
• It is seen over a wide age range (1st-7th
  decades).
• There is a slight female gender predilection.
• It occurs most commonly in the parotid gland
  where it often appears as an asymptomatic
  swelling. High grade tumors may cause pain or
  facial nerve palsy.

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Mucoepidermoid Carcinoma Clinical Features

• Minor salivary glands represent the second most
  common location of occurrence with the palate
  being the most common site among these.
• Mucoepidermoid carcinomas of the minor glands
  tend to appear as asymptomatic swellings, which
  may be fluctuant and have a blue/red color.
• Although uncommon, other oral sites include the
  lower lip, floor of mouth, tongue and retromolar
  pad areas. The lesion can also occur
  intraosseously.

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Mucoepidermoid Carcinoma Cause and Treatment

• The cause of mucoepidermoid carcinoma is unknown.
• The treatment is predicated by location,
  histopathologic grade and clinical stage.
• The treatment of choice is surgical excision with
  high grade tumors requiring a wider resection.
  Radical neck dissection is carried out on
  patients with clinical evidence of metastatic
  disease and considered for those with large or
  high-grade tumors.
• Postoperative radiation therapy is used with
  aggressive tumors.

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Mucoepidermoid Carcinoma Prognosis and
Significance

• Low-grade tumors may clinically resemble
  mucoceles.
• Prognosis depends upon the grade and stage of the
  tumor.
• Low-grade lesions have approximately a 90 cure
  rate.
• High-grade tumors have approximately a 30 cure
  rate.
• Prognosis is better in children overall.
• Prognosis is poorer with tumors occurring in the
  submandibular glands or at the base of the tongue.

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Mucoepidermoid Carcinoma Comparing Two Grading
Systems

• AUCLAIR ET AL. (1992)
• Parameter Point Value
• Intracystic component lt 20
• 2
• Neural invasion present
• 2
• Necrosis present
• 3
• Four or more mitoses per 10 high-power fields
• 3
• Anaplasia present
• 4
• Grade Total Point Score
• Low 04
• Intermediate 56
• High 714

• BRANDWEIN ET AL. (2001)
• Parameter Point Value
• Intracystic component lt25
• 2
• Tumor front invades in small nests and islands
• 2
• Pronounced nuclear atypia
• 2
• Lymphatic or vascular invasion
• 3
• Bony invasion
• 3
• Greater than four mitoses per 10 high- power
  fields
• 3
• Perineural spread
• 3
• Necrosis
• 3

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Intraosseous Mucoepidermoid Carcinoma (Central)

• This malignant neoplasm is more common in
  middle-aged adults and has a female gender
  predilection.
• It occurs more commonly in the mandible (31) in
  the molar-ramus region.
• Most often presents as a cortical swelling
  although some are discovered incidentally.

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Intraosseous Mucoepidermoid Carcinoma (Central)

• Pain, trismus and paresthesia may occur.
• On x-ray the lesion usually presents as a
  unilocular or multilocular radiolucency with
  well-defined borders.
• Occasionally, it is associated with an unerupted
  tooth.

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Central Mucoepidermoid Carcinoma Cause,
Treatment Significance

• The cause is unknown. Does it arise in ectopic
  salivary gland, from maxillary sinus lining or
  odontogenic epithelium?
• Radical surgery offers the best chance for cure.
• Most central mucoepidermoid carcinomas are
  low-grade tumors.
• Approximately, 10 of the patients die as the
  result of the disease, most due to local
  recurrence.
• Some tumors are misdiagnosed.

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Acinic Cell Adenocarcinoma Clinical Features

• This malignant tumor is most common in the
  parotid gland it accounts for from 2-4 of all
  minor salivary gland tumors.
• The buccal mucosa, lips and palate are the most
  common minor salivary gland sites.
• There is a wide age range with a mean in the 40s.

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Acinic Cell Adenocarcinoma Clinical Features

• Sixty percent of the patients are females.
• The tumor typically presents as an asymptomatic,
  slow-growing mass.
• Occasionally, pain and tenderness are presenting
  features.
• Facial nerve paralysis is infrequent but an
  ominous sign for these malignant parotid tumors.

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Acinic Cell Adenocarcinoma Cause and Treatment

• The cause is unknown.
• In the parotid, it is best treated by lobectomy
  while at other sites surgical excision is the
  method of choice.
• Lymph node dissection is only carried out if
  tumor is present.
• Radiation therapy is used for uncontrolled
  disease.

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Acinic Cell Adenocarcinoma Prognosis and
Significance

• This tumor has one of the better prognoses
  overall of any malignant salivary gland tumor.
• Tumors of the minor glands have a better
  prognosis than those of the major glands.
• 6-26 of the patients die as the result of their
  tumor.
• It is considered a low-grade malignancy.

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Malignant Mixed Tumors

• Carcinoma Ex Pleomorphic Adenoma
• Carcinoma Ex Mixed Tumor
• Carcinosarcoma
• Metastasizing Mixed Tumor

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Malignant Mixed Tumors

• The most common of these is the carcinoma ex
  pleomorphic adenoma, which is characterized by
  malignant transformation of the epithelial
  component of a previously benign pleomorphic
  adenoma.
• The carcinosarcoma is a rare mixed tumor in
  which both carcinomatous and sarcomatous elements
  are present.
• The metastasizing mixed tumor has histopathologic
  features that are identical to the common
  pleomorphic adenoma but the lesion metastasizes.

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Malignant Mixed Tumors Clinical Features

• Carcinoma ex pleomorphic adenoma arises in a
  pleomorphic adenoma.
• It occurs in patients about 15 years older than
  those with a pleomorphic adenoma.
• Eighty percent occur in the major glands while
  2/3 of the intraoral tumors arise in the palate.

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Malignant Mixed Tumors Clinical Features

• There is a slight female gender predilection.
• Pain, recent rapid growth, ulceration, fixation
  and facial nerve palsy are common features of
  those tumors arising in the parotid gland.
• Some intraoral tumors remain indistinguishable
  from their benign counterparts.
• Carcinosarcomas and metastasizing mixed tumors
  are quite rare and thus there is little reliable
  clinical data.

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Malignant Mixed Tumors Cause, Treatment,
Prognosis Significance

• The cause is unknown for these tumors.
• Treatment consists of wide surgical excision
  often with lymph node dissection and adjunctive
  radiation therapy.
• The overall prognosis for these tumors is roughly
  50-50.

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Adenoid Cystic Carcinoma

• This is one of the more common and best
  recognized salivary gland malignancies.
• An old term for this lesion is cylindroma. It
  should be avoided today because a skin adnexal
  tumor has been given the same terminology.

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Adenoid Cystic Carcinoma Clinical Features

• Approximately, 50 of these tumors develop in
  the minor salivary glands with the palate being
  the most common site.
• It is more common in middle-aged patients and has
  a slight female gender predilection.
• Adenoid cystic carcinoma usually presents as a
  slow-growing mass and pain is a common feature.
  The pain is often described as constant,
  low-grade and dull.

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Adenoid Cystic Carcinoma Clinical Features

• In parotid tumors facial nerve paralysis may
  occur.
• Palatal tumors may be smooth or ulcerated.
• Palatal tumors or those of the maxillary sinus
  may show radiographic evidence of bony
  destruction.

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Adenoid Cystic Carcinoma Cause and Treatment

• The cause is unknown.
• Surgical excision is usually the treatment of
  choice and adjunct radiation therapy may slightly
  improve prognosis.

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Adenoid Cystic Carcinoma Prognosis and
Significance

• Perineural invasion is highly characteristic but
  said to have little effect on prognosis.
• Late recurrence and metastasis make 5-year
  survival rates of little significance.
• Tumors with a solid histopathologic pattern have
  the worst prognosis all other things being equal.
• Tumors of the maxillary sinus and submandibular
  gland have poor prognoses relative to site.
• Death, if it results from tumor, occurs because
  of local recurrence or distant metastases.

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Polymorphous Low-Grade Adenocarcinoma

• Lobular Carcinoma
• Terminal Duct Carcinoma

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Polymorphous Low-Grade Adenocarcinoma Clinical
Features

• This malignant salivary gland neoplasm occurs
  almost exclusively in the minor salivary glands,
  where it is one of the more common malignancies.
• Sixty percent of the cases occur on the hard or
  soft palates following in frequency by the upper
  lip and buccal mucosa.
• Most commonly occurs in older adults (6-8th
  decades) and there is a female gender
  predilection ( two thirds of cases).
• Most commonly presents as a slow-growing mass
  occasionally accompanied by bleeding or
  discomfort.

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Polymorphous Low-Grade AdenocarcinomaCause
Treatment

• The cause of this tumor is unknown.
• Treatment consists of wide surgical excision
  sometimes including resection of the underlying
  bone.
• It is a low-grade malignancy which uncommonly
  metastizes.
• Radical neck surgery is usually unwarranted.

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Polymorphous Low-Grade Adenocarcinoma Prognosis

• Eighty percent of the patients are tumor free
  after treatment and most of the rest are
  controlled with re-excision.
• Death due to the tumor is rare.
• Perineural invasion, like adenoid cystic
  carcinoma, occurs frequently and does not appear
  to affect prognosis if it occurs.
• This tumor must be differentiated from adenoid
  cystic carcinoma.
• The histopathology of this tumor is deceptively
  uniform and can be mistaken for a benign lesion.

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Salivary Adenocarcinoma, NOS

• These tumors are malignant salivary gland
  neoplasms that cannot be further classified as
  one in the existing classification schemes, hence
  NOS (not otherwise specified)
• Since these adenocarcinomas represent such a
  diverse group it is difficult to generalize
  either about their clinical or microscopic
  features.

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Salivary Adenocarcinoma, NOS

• They may present as asymptomatic masses or cause
  pain and nerve paralysis.
• Their prognosis is guarded by patients with
  early-stage, well-differentiated tumors have a
  better outcome. Oral tumors generally have a
  better prognosis than those occurring in the
  major glands.