Salivary gland disorders, pathology and presentation Flashcards

1
Q

What is aplasia?

A

failure to develop normally
- aplasia of salivary glands is very rare. It may occur as an isolated event or as part of a hereditary syndrome

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2
Q

What is atresia?

A

failure to be tubular
- atresia of ducts is also very uncommon

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3
Q

What duct is most often affected when atresia does occur?

A

submandibular duct

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4
Q

What is a mucocoele?

A

a cystic cavity filled with mucous

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5
Q

What are the 2 types of mucocoele?

A
  • extravasation mucocoele (also called mucous extravasation cyst)
  • retention mucocoele (also called mucous retention cyst)
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6
Q

How does an extravasation mucocoele present?

A

as a bluish or transparent swelling

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7
Q

Where do extravasation mucocoeles most commonly affect?

A

minor glands especially in the lower lip

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8
Q

What age range do extravasation mucocoles occur in?

A

a wide age range but most frequently seen in children and young adults

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9
Q

Are extravasation mucocoeles symptomatic?

A

no, asymptomatic

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10
Q

What is typically associated with extravasation mucocoeles?

A

typically history of trauma associated with the lesion

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11
Q

What causes an extravasation mucocoele?

A
  • due to ruptures duct with leakage of saliva into surrounding connective tissue
  • leaked saliva elicits an inflammatory reaction
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12
Q

What is the histopathology of a mucous extravasation cyst?

A
  • lesion appears as a cystic cavity filled with mucin in connective tissue
  • mucin is surrounded by inflamed granulation tissue, typically with lots of macrophages
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13
Q

Why are mucous extravasation cysts not classed as true cysts?

A

no epithelial lining

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14
Q

What is the treatment of extravasation mucocoeles?

A

removal of all of the mucocoele (excision) together with the associated ruptured duct and gland where possible to prevent recurrence

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15
Q

What is the appearance of a mucous retention cyst?

A

similar appearance to a extravasation cyst, but less common
(a bluish or transparent swelling)

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16
Q

What can mucous retention cysts affect?

A

can affect the major and minor glands
- rare on lower lip

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17
Q

What does a mucous retention cyst represent?

A

represents cystic dilation of a duct typically due to obstruction

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18
Q

What is the histopathology of a mucous retention cyst?

A
  • mucin retained within a dilated duct
  • cyst lining is epithelial lining of the duct
  • as saliva is retained within the duct and doesn’t escape, there is much less inflammation
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19
Q

Why is there less inflammation from a mucous retention cyst than from a extravasating cyst?

A

it is the leaked saliva that causes inflammation
- saliva does not leak from a mucous retention cyst but does from an extravasating cyst

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20
Q

What is the treatment for a mucous retention cyst?

A

excision

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21
Q

How does a ranula present?

A
  • presents as a painless soft bluish swelling in floor of mouth
  • unilateral
  • 2-3cm in size
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22
Q

What causes a ranula?

A

it is an uncommon form of mucous extravasation cyst arising from sublingual gland

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23
Q

What is a ‘plunging ranula’?

A

arises when the mucin passes through and develops below mylohyoid as swelling in neck

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24
Q

What is the treatment of a ranula?

A

drainage of the cystic cavity and removal of sublingual gland

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25
Q

What is sialadenitis?

A

inflammation of salivary glands

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26
Q

What are the most common causes of sialadenitis?

A

bacterial or viral infection

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27
Q

What gland is most often affected by acute bacterial sialadenitis?

A

parotid

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28
Q

What is a major predisposing factor of acute bacterial sialadenitis?

A

decreased salivary flow

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29
Q

How do patients with acute bacterial sialadenitis present?

A
  • with pain, swelling, tenderness, exsudation of pus
  • there may be redness of overlying skin
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30
Q

What are bacteria frequently associated with acute bacterial sialadenitis?

A

S. aureus, streptococci, and oral anaerobes

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31
Q

What is the treatment for acute bacterial sialadenitis?

A

appropriate antibiotics after culture/sensitivity testing

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32
Q

What is chronic bacterial sialadentitis usually secondary to?

A

duct obstruction

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33
Q

What is duct obstruction more frequently caused by?

A

stones/salivary calculi/mucous plus (parotid)

34
Q

What gland is most often affected by chronic bacterial sialadenitis?

A

submandibular

35
Q

What are the symptoms of chronic bacterial sialadenitis?

A

may be asymptomatic or may be intermittent painful swelling which is usually mealtime related
- typically unilateral

36
Q

What gland is most commonly affected by salivary calculi?

A

submandibular

37
Q

What is salivary calculi caused by?

A

mineralisation of phosphates from supersaturated saliva being deposited around a central industry of cell debris

38
Q

Where may calculi form?

A

within ducts in the gland or in the main excretory duct
- mainly occur in adults

39
Q

What is the appearance of salivary calculi?

A

calculi vary in size, may be more than one, may be round or ovoid, rough or smooth, and usually yellowish in colour

40
Q

What often grows on the surface of salivary calculi?

A

bacteria, which can elicit an inflammatory response

41
Q

What are the symptoms of salivary calculi?

A
  • no symptoms until stone causes obstruction
  • typically unilateral swelling/pain, often at mealtimes
42
Q

Do salivary calculi cause dry mouth?

A

no, however factors such as dry mouth and dehydration can predispose someone to stones

43
Q

What does treatment of salivary calculi depend on?

A

treatment varies depending on size and location of stones

44
Q

What are the treatment options for salivary calculi?

A
  • may be possible to remove or breakdown some stones
  • alternatively, it may be necessary to remove the gland, especially if it has become very damaged by longstanding infection
45
Q

What is the histopathology of chronic bacterial sialadenitis?

A
  • the salivary acini become atrophic and are replaced by fibrous scar tissue
  • the salivary ducts within the gland become dilated and there is often hyperplasia of the duct epithelium
  • a chronic inflammatory infiltrate with predominantly plasma cells and lymphocytes is seen within the gland
46
Q

What may progressive chronic inflammation result in?

A

almost complete replacement of salivary parenchyma by fibrous tissue. This can result in a firm mass within the gland which may be mistaken for a neoplasm

47
Q

What is the treatment for chronic bacterial sialadenitis?

A
  • the gland may recover from mild sialadenitis if the associated obstruction can be removed
  • if more extensive sialadenitis, the obstruction and gland requires to be excised
48
Q

What is mumps (viral sialadenitis)?

A
  • acute, contagious infection caused by paramyxovirus
  • spreads via saliva
49
Q

What does mumps cause?

A
  • painful swelling of parotids and other exocrine glands
  • patients also present with fever, headache and malaise
50
Q

What are some significant complications of mumps?

A

orchitis, oophoritis, nephritis

51
Q

What is HIV-associated salivary gland disease?

A

may be the first clinical sign of HIV infection

52
Q

What does HIV-associated salivary gland disease cause?

A
  • swelling of major glands (most frequently parotid) and may be bilateral
  • gland swelling is occasionally painful and soft to palpate
  • multiple cysts are seen on imaging of the glands
53
Q

What does the histopathology of HIV-associated salivary gland disease show?

A
  • histopathology of glands shows multiple large cysts and dense lymphoid tissue
  • the features are suggestive but NOT definitively diagnostic
54
Q

What are the clinical features of necrotising sialometaplasia?

A
  • more common in males than females
  • more frequently seen in older patients and smokers
  • mainly affects minor salivary glands, especially those in the hard palate
  • presents as a large, deep ulcer
  • may be painful
  • slow to heal, often takes several weeks
55
Q

What is the aetiology of necrotising sialometaplasia?

A

uncertain but it likely arises due to ischaemia or infarction secondary to trauma

56
Q

Why are biopsies of necrotising sialometaplasia often taken?

A

the appearance of the slow healing ulcers are suspicious for oral cancer or a salivary gland cancer

57
Q

What does the histopathology of necrotising sialometaplasia show?

A
  • features include necrosis of salivary acini, inflammation and hyperplasia/metaplasia of salivary ducts
  • the changes in the duct epithelium can be mistaken for cancer too
58
Q

What is the treatment for necrotising sialometaplasia?

A

none required, slowly resolves itself, biopsy is usually curative

59
Q

What is Sjögren’s syndrome?

A

an autoimmune disease of unknown cause characterised by lymphocytic infiltration and acinar destruction of lacrimal and salivary glands (and other exocrine glands)

60
Q

How do patients with Primary Sjögren’s syndrome present?

A

patients have dry eyes and/or a dry mouth with no associated connective tissue disease

61
Q

How do patient’s work Secondary Sjögren’s syndrome present?

A

patients have dry eyes and/or a dry mouth and a connective tissue disease e.g. Rheumatoid arthritis

62
Q

Who does Sjögren’s syndrome affect?

A
  • onset of Sjögren’s syndrome is in middle age
  • females are much more commonly affected than males
63
Q

What do systemic symptoms of Sjögren’s syndrome include?

A

fatigue, joint pain, peripheral neuropathy

64
Q

What are the complications of dry mouth (caused by Sjögren’s syndrome)?

A

caries, periodontal disease, difficulty with swallowing, speech, taste, predisposition to infection

65
Q

Other than dry mouth, what issues can be caused by Sjögren’s syndrome?

A
  • there may be swelling of salivary glands, esp. parotids
  • eye problems due to dry eyes
  • connective tissue disease in secondary Sjögren’s syndrome
  • patients with primary sjögren’s syndrome have an increased risk of developing lymphoma in affected glands
66
Q

What is sialedenosis?

A
  • a non-inflammatory, non-neoplastic, bilateral, symmetrical swelling of salivary glands
  • predominantly parotid glands affected
  • painless
67
Q

What is sialedenosis associated with?

A

malnutrition, anorexia, bulimia, alcoholism, diabetes mellitus, certain drugs and hormonal disturbances

68
Q

What does sialadenosis result in?

A

hypertrophy of serous acini

69
Q

What percentage of salivary gland tumours account in the minor salivary glands?

A

15-20%

70
Q

What percentage of major salivary gland tumours occur in the parotid?

A

90%

71
Q

What percentage of minor salivary gland tumours arise in the palate?

A

55%

72
Q

What percentage of minor salivary gland tumours arise in the upper lip?

A

20%

73
Q

What methods of biopsy can be used in the diagnosis of salivary gland tumours?

A
  • fine needle aspiration (FNA)
  • core biopsy
  • open biopsy
  • excision
74
Q

What are the current categories in the WHO classification of salivary gland tumours?

A
  1. malignant tumours
  2. benign tumours
  3. non-neoplastic epithelial lesions
  4. benign soft tissue lesions
  5. haematolymphoid tumours
75
Q

What is mucoepidermoid carcinoma?

A
  • the most common of the 22 primary epithelial salivary gland malignant tumours in the WHO classification
  • it is locally invasive, it can recur and can metastasise
  • up to 80% have MAML2 gene fusions
76
Q

What and who does mucoepidermoid carcinoma affect?

A
  • more frequently affects the parotids but can occur in others including minor glands
  • can be seen in children and young adults
  • F>M
77
Q

What is the most common type of ALL salivary gland tumours?

A

pleomorphic adenoma

78
Q

What percentage of all parotid tumours does pleomorphic adenoma account for?

A

approx. 60%

79
Q

When is the peak incidence of pleomorphic adenoma?

A

5th and 6th decades, but can occur at any age

80
Q

How does pleomorphic adenoma present?

A
  • benign, painless, slow growing, ‘rubbery’ lump
  • usually solitary although recurrences may be multifocal
  • most are associated with gene rearrangements in PLAG1 or HMGA2