View source for Non neoplastic disorders of salivary glands



[[Category:Laryngology]]

Introduction:


 

A wide range of non neoplastic disorders are known to affect the salivary glands.
 
The common among them include:


 

Mumps


Acute suppurative sialadenitis


Sjogren syndrome


Drug induced xerostomia


 

WHO Classification of non-neoplastic salivary gland disorder:


 

Tumor like lesions:


 

1. Sialadenosis


2. Oncocytosis


3. Necrotising sialometaplasia


4. Benign lymphoepithelial lesion


5. Salivary gland cysts


6. Chronic sclerosing sialadenitis


 

Congenital salivary gland disease:


 

Agenesis of salivary glands:


 

Agenesis of major salivary glands is a very rare even, with only a few cases reported.
 This congenital anomaly may caue profound xerostomia in children.  Oral cavity sequelae
include dental caries, candidiasis and ascending sialadenitis.  Parotid gland is
more often involved.  Parotid gland agenesis is usually bilateraly and may also be
associated with abnormalities of head and neck region.


This condition may also be associated with ectodermal defects that include:


Hypohidrotic ectodermal dysplasia


Lacrimal-auriculo-dento-digital anomaly.


 

Congenital imperforate submandibular salivary gland duct:


 

This could cause unilateral cystic swelling in the floor of the mouth as a result
of imperforate submandibular gland ducts.  This is postulated to be caused by congenital
failure of canalization of the terminal end of the duct.  This condition responds
to simple incision and decompression of the fluid filled duct.


 

Infections of salivary glands:


 

Viral and bacterial infections can frequently affect the salivary glands.  It should
be pointed out that fungal infections involving salivary glands are extremely rare.


 

Epidemic parotitis (Mumps):


 

This is an acute, contagious, viral disease that cause painful enlargement of the
salivary gland, commonly the parotids.  This condition could also lead to orchitis,
meningoencephalitis and pancreatitis.  Diagnosis is usually made on clinical grounds.
 


 

Laboratory diagnosis of mumps is not necessary and is indicated only in the following
scenario:


 

1. Unilateral parotid involvement


2. Recurrent parotitis


3. When parotid gland enlargement occurs in previously immunized patients


4. When it causes prominent involvement of tissues other than the salivary glands


RT-PCR is the preferred method of diagnosis.


 

Spread of infection is via droplets.  People with mumps are most contagious during
the first 2 days before symptoms appear.  Patients can also continue transmitting
infections even one week after complete alleviation of symptoms.


 

Treatment:


 

This is purely supportive.  The affected child should be isolated until the glandular
swelling and pain subsides.  Soft diet that involves minimal chewing is advisable.
 Acidic substances like the citrous juices that could cause worsening of the pain
must be avoided.


Mumps can be prevented by vaccination.  Mumps vaccine has been included in the universal
immunization program.


Some of the complications of Mumps include:


1. Deafness


2. Orchitis


3. Pancreatitis


 

Acute suppurative sialadenitis:


 

This is an uncommon disorder characterized by painful swelling and purulent discharge
from the duct of the affected gland.  There is also associated dysgeusia and cervical
adenopathy.  In severe cases there could be associated pyrexia, malaise.  There is
always an associated risk of abscess formation, parapharyngeal space infection and
Ludwig's angina.


This condition can affect both children and adults.  Parotid gland is the common
major salivary gland that could be involved.  This condition is highly uncommon in
neonates. Immunodeficiency and other concurrent illness could predispose to childhood
suppurative parotitis.  Long standing xerostomia caused by irradiation can cause
suppurative parotitis in adults.  Ductal obsturction either by sialolithiasis / malignancy
/ FB may also predispose to suppuration of parotid gland.


 

Common pathogens isolated:


 

Staph aureus and different anaerobic bacteria have been isolated.  Gram negative
pathogens have been isolated in hospitalized patients.


Diagnosis of suppurative sialadenitis is usually based on the history and clinical
features.  Microbial culture of pus could reveal the causative organism.


 

Management:


 

Include manitenance of hydration and administration of parenteral antibiotics.  Commonly
used antibiotics include penicillinase resistant antibiotis (flucloxacillin, amoxycillin
clavulanic acid combination).


Surgical drainage should be considered if there is lack of improvement after 3-5
days of antibiotic therapy.  Superficial parotidectomy should be performed if the
disease is recurrent / chronic.


 

Chronic recurrent sialadenitis:


 

This condition commonly involves parotid gland.  This is due to recurrent bacterial
infection.  During acute exacerbation parotid gland is enlarged and tender.  Pus
can be expressed from its duct.  Between acute episodes the gland is firm and slightly
enlarged.  Culture of pus from the duct would reveal staphylococci / streptococci.


 

Sialadenosis:


 

This is a non neoplastic, non inflammatory enlargement of salivary glands.  This
condition involves mainly the parotid gland.  The enlargement is bilateral.  There
may be associated recurrence or pain. This disease is due to increased weight, improper
nutrition, alcoholic cirrhosis, hypothyroidism.


 

Classification of sialadenosis:


 

1. Hormonal sialadenosis


2. Sex hormonal


3. Diabetic


4. Thyroid


5. Neurohormonal sialadenosis


6. Malnutritional sialadenosis


7. Mucoviscidosis


8. Drug induced sialadenosis


 

Clinical features:


 

This condition is characterised by the presence of chronic, afebrile salivary gland
enlargement usually of the parotid gland.  The enlargement is slowly evolving, indolent
and recurrent.  There is decreased amount of salivary secretion.  Analysis of saliva
demonstrates increased levels of potassium and decreased levels of sodium.  There
is also associated hypertrophy of acinar cells which crowd and compresses the finer
terminal ducts.  This forms a leafless tree pattern.  The acinar cells hypertrophy
is so pronounced that it could be three times greater than its normal size.  The
nuclei of the acinar cells are found to be displaced to the cell base and the cytoplasm
is engorged with zymogen granules.  


In patients with long standing diabetes, there may be acinar atrophy and fatty infiltration.


Subtotal parotidectomy is the ideal management modality in these patients.


 

HCV related sialadenitis:


 

Hepatitis C viral infection frequently gives rise to a wide spectrum of extra-hepatic
manifestation.  This includes salivary gland disease. HCV infected individuals may
have some salivary / lacrimal abnormality.  HCV may present in the saliva of majority
of patients. 


 

Necrotizing sialometaplasia:


 

This is a benign self limiting rare inflammatory disease which mimics malignant neoplasm.
 Another name to describe this condition is salivary gland infarction.  Clinical
and histopathological features could sometimes simulate squamous cell carcinoma /
mucoepidermoid carcinoma.


 

Etiology:


 

1. Associated with history of trauma / RT / Surgery


2. Mostly related to vascular ischemia


3. Tobacco use has been suggested to be a risk factor


 

Clinical features:


 

These lesions are usually painless, and sometimes less frequently could cause pain
and numbness.  These lesions are characterized by swelling with or without ulceration
in anatomic sites.  Typically these lesions show crateriform ulcer of the palate
that simulate malignant process.


 

Histopathology:


 

Is characterized by acinar necrosis in early lesions to be followed by associated
squamous metaplasia of salivary ducts.  The lobular architecture of the gland is
preserved in all cases (this is a vital histologic clue).


Biopsy of the lesion is a must to rule out malignancy.


These lesions usually resolve on their own with a healing time of 5-6 weeks.


 

Sjogrem's syndrome:


 

This is the second most common auto immune disorder.  This is a chronic systemic
autoimmune disorder that principally involves salivary and lacrimal glands resulting
in xerostomia and xeropthalmia. In the eye keratoconjunctivitis sicca is produced.


 

Two forms of this disease could manifest:


Primary Sjogren's syndrome:


This is sicca only syndrome


 

Secondary Sjorgren's syndrome:


 

In addition to the sicca syndrome, autoimmune diseases are seen.


HLA antigens are found with great frequency in patients with Sjogren's syndrome.
 HLA -DRW52 is associated with both forms of Sjogren's syndrome.  HLA -B8 and HLA
-DR3 are seen in increased frequency in the primary form of the disease.


 

Pre valance:


 

This condition is prevalent in 0.5% of the population.  Females out number males
by a ratio of 9:1.  Secondary Sjogren's syndrome is associated with rhematoid arthritis
and systemic lupus erythematosus.


 

Clinical features:


 

Principal symptom is xerostomia (dry mouth).  These patients complain of difficulty
in swallowing, altered taste sensation, and difficulty in wearing dentures.  The
tongue becomes fissured with atrophy of the papillae.  Firm enlargement of salivary
glands occurs during the phase of the disease.  The swelling could be unilateral
/ bilateral.  Swelling is non painful and intermittent in nature.   Sialography reveals
punctate sialectasia and lack of normal arborization of ductal system showing fruit
laden tree pattern.


Keratoconjunctivitis sicca, a prominent feature is not only reduced tear production
by lacrimal glands but also pathological effects on the epithelial cells on the ocular
surface.  Schirmer test proves this condition.


 

Histopathology:


 

Lymphocytic infiltration of salivary glands with destruction of acinar units.


 

Management:


 

Dry eyes can be managed by periodic use of artificial tears.  Artificial saliva /
use of sugarless candy helps to keep mouth moist.


Sialogogue like pilocarpine can be used to increase salivary flow.


 

Mikulicz disease:


 

This is also known as benign lympho-epithelial lesion.  This condition have bilateral
painless swelling of lacrimal glands and all the salivary glands.


This condition is most common in females.  It affects adult individuals.  


Histology reveals infiltration of salivary gland tissue destroying / replacing the
acini with the persistence of islands of epithelial cells.


Biopsy from the swollen glands is the key to diagnosis of Mikulicz disease.  Treatment
is purely symptomatic.


 

Sialolithiasis:


 

Also known as salivary duct stone / salivary duct calculus.


These are deposition of calcium salts around a central nidus which may consist of
altered salivary mucins, desquamated epithelial cells, bacteria, foreign bodies,
or products of baterial decomposition.


This is the most common disorder of salivary gland.  Patients with sialolithiasis
involve duct of major salivary glands. These patients complain of severe pain particularly
just before and after meals owing to stimulation of salivary flow associated with
swelling of salivary gland.


This condition can occur at any age, but is common in middle aged adults.  Distribution
of sialolithiasis is as follows:


Parotid - 20%


Submandibular gland - 64%


Sublingual gland - 16%


Microscopically calcified mass exhibits concentric laminations around a central nidus
of amorphous debris.  This lamellated structure of the calculus is a result of successive
deposition of inorganic and organic material.  Associated periductal inflammation
could also be seen.


 

Management:


 

Small calculi can be removed by manipulation or increasing the salivation by sucking
a lemon which causes expulsion of the stone.  Inflammation and infection can be managed
by intravenous administration of antibiotics.  Larger stones need surgical exposure
for removal.  Lithotripsy has been advocated in some cases.


Drug induced salivary gland disorders:


Wide ranging drug related salivary gland disorders are possible.  Salivary glands
increase in size with associated xerostomia.  Salivary gland pain has been rarely
associated with guanethidine therapy.  Other drugs that can cause these problems
include Bretylium, clonidine, methyldopa and some cytotoxics.


Painless, bilateral salivary gland enlargement may be an adverse reaction of phenylbutazone,
chlorhexidine, epinephrine, naproxen and clozapine.  Mild acute sialadenitis (iodide
mumps) can arise in patients after imaging procedures using iodine based contrast
medium.  These patients usually present with painless bilateral parotid / submandibular
salivary gland swellings.  These swellings are rapid in onset and gradually disappear
during the course of one week. Radio active I 131 used in the treatment of thyroid
cancers bones in on the salivary glands.  Iodine is concentrated in the saliva and
is secreted.  This causes a certain amount of damage to the salivary gland parenchyma
causing swelling and pain.  This is more common in parotid gland.  Symptoms may develop
immediatly after its use or months later.  Adequate hydration will help to mitigate
this problem.


 

Juvenile recurrent parotitis:


 

This condition is defined as recurrent parotid inflammation, generally associated
with non-obstructive sialectasis of parotid gland.  The usual age of onset is 3-6
years.  Childhood disease is more common in males, while adult onset disease normally
arises in females.  Number of attacks could vary between 10-20 / year.  This disease
is characterized by local pain, swelling that could last up to 2 weeks and could
resolve spontaneously.  Fever and redness are also common and occasionally a drop
of pus may be expressed from the parotid duct. Sialography and ultrasound can reveal
sialectasis, which is bilateral.


Pathogenesis remains unknown.  These patients have a low salivary rate.  Histopathology
reveals periductal lymphocytic infiltration.  Conservative treatment is ideal.


 

Pneumoparotitis:


 

This is another rare cause of parotid gland enlargement, caused by reflux of air
through stenson's duct into the acini of the gland with subsequent dilatation.  This
condition could occur as a transient / recurrent phenomenon.  Recurrent insufflation
is not entirely benign and may predispose to sialectasis, recurrent parotitis and
even subcutaneous emphysema.  This condition is more common in wind instrument players,
balloon and glass blowers or any individual who increase intraoral pressure by forcefully
blowing up the cheeks consciously or as a habit.


There is no need for investigation as the history is often revealing.  Sialography,
ultrasound, CT, MRI can be used as diagnostic tools.


Management is conservative.  Avoiding increase in intraoral pressure could help.


 

Kuttner tumor / Chronic sclerosing sialadenitis:


 

In 1896 Kuttner reported four cases of this condition.  They presented as indurations
of submandibular gland.  Descriptively this condition is known as chronic sclerosing
sialadenitis.  This is a chronic disorder of the salivary gland.


Clinically these lesions appear as firm swelling of the glands and may be difficult
to differentiate from salivary gland neoplasia.  Submandibular gland is more commonly
affected, sometimes parotids, sublingual and minor salivary glands can also be affected.


Histologically, these lesions are characterized by progressive periductal sclerosis,
acinar atrophy and gland infiltration by lymphocytes. The pattern of distribution
of these lymphocytes suggests an immunologic reaction.  Sialoliths have also been
found in nearly a quater of these patients.


 

Xanthogranulomatous sialadenitis:


 

This is a rare disorder manifesting as solitary swelling in a major salivary gland.
 Xanthogranulomatous inflammation typically occurs as a type of pyelonephritis of
the kidney.  


Histologically these lesions appear as sheets of cells with clear foamy cytoplasm
and small bland nuclei admixed with neutrophils, lymphocytes, plasma cells and mast
cells.  It has been suggested that this condition could reflect underlying non-Hodgekin's
lymphoma.