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HEAD AND NECK

Diseases of the salivary


glands

The submandibular gland produces mixed mucinous and serous saliva, accounting for the majority of the saliva at rest. The
gland lies between the mandible superiorly, the anterior belly of
the digastric muscle antero-inferiorly and the posterior belly of
digastric postero-inferiorly. The gland is divided into superficial
and deep lobes as it hooks around the posterior border of the
mylohyoid muscle. Superficially the gland is covered by the deep
layer of investing cervical fascia The mandibular and cervical
branches of the facial nerve lie on this. The deep lobe lies on the
hyoglossus muscle medially, with the lingual nerve positioned
superiorly and the hypoglossal nerve inferiorly. Whartons duct
runs anteriorly to open into the oral cavity lateral to the fraenulum of the tongue.

Paula Bradley
James OHara

Abstract
Salivary gland disease is managed by a number of specialities. There are
three pairs of major salivary glands and several hundred minor salivary
glands within the upper aerodigestive tract. Pathology is diverse,
including infective, inflammatory and neoplastic diseases. Clinical presentation is usually with a lump within, or swelling of the gland. Investigations combine clinical assessment, fine needle aspiration cytology and
radiology. Management is medical or surgical dependent on pathology.
Surgical intervention is commonly performed for chronic inflammatory
disorders and neoplasms. Management requires a sound knowledge of
anatomy and oncologic principles.

The sublingual glands lie deep to the mucosa of the floor of the
mouth between the mylohyoid and genioglossus muscles,
opening directly onto the mucosa, or into the submandibular
ductal system. They produce mucinous saliva.

Clinical assessment

Keywords Inflammatory diseases; minor salivary glands; neoplasia;

For the majority of patients, the history helps establish a diagnosis. It is important to determine chronicity, whether pain is a
feature, fluctuation or change in size with eating with a differential diagnosis. A history of chronic inflammatory disorders is
important. Palpation of the lump should be performed bimanually with a gloved finger intraorally. The facial nerve function
should be assessed, the oropharynx should be examined to
establish any deep lobe extension and the cervical lymph nodes
examined for any associated lymphadenopathy.

parotid gland; submandibular gland

Diseases of the salivary glands are heterogeneous and may present to a number of specialities. The usual presentation of a
lump, in or making up all of a gland, may indicate localized
pathology or be part of a more generalized condition. The salivary glands are the paired parotid, submandibular and lingual
glands along with several hundred minor salivary glands,
distributed throughout the upper aerodigestive system.

Imaging
Plain radiographs have an historic role in the investigation of
salivary gland pathology, predominantly in the diagnosis of
submandibular duct calculi, 90% of which are radio-opaque.
Sialography is the most sensitive investigation to assess
ductal pathology but is increasingly being replaced by magnetic resonance sialography and ultrasound. Ultrasound (US) is
increasingly the initial imaging modality of choice.1 US allows
simultaneous fine needle aspiration cytology (FNAC)
(Figure 1), will pick up 90% of salivary duct stones and can
characterize salivary tumours in great detail but is limited by
the mandible in obtaining good views of the deep parotid lobe.
Computed tomography (CT) is more accessible and cheaper
than magnetic resonance imaging (MRI), but images can be
distorted by dental artefact. It is also useful in imaging bone
involvement and the thorax and the abdomen, in the case of
metastatic disease from and to the salivary glands. MRI gives
superior soft tissue images and clearer definition of the
anatomical relations to cranial nerves and peri-neural spread
(Figure 2).

Anatomy
The parotid gland is the largest of the salivary glands producing
mainly serous saliva. It covers the area anterior to the tragus of
the external ear from the zygomatic arch superiorly to the upper
neck inferiorly. It is shaped like a wedge, lying between the
ramus of the mandible anteriorly and the temporal bone posteriorly. Its deep lobe occupies the pre-styloid component of the
parapharyngeal space and approaches the lateral wall of the
oropharynx. The parotid (Stensen) duct crosses the masseter,
piercing the buccinator opening into the oral cavity opposite the
second upper molar tooth.
The facial nerve enters the parotid gland, dividing into two
main divisions and five branches (temporal, zygomatic, buccal,
mandibular and cervical), splitting the parotid gland into its
superficial and deep lobes. The nerve is motor to the muscles of
facial expression, sensory to a small patch of the external ear
canal and special sensory to the anterior two-thirds of the
tongue.

Fine needle aspiration cytology (FNAC)


Paula Bradley MRCS is a Specialty Trainee in the Northern Deanery, UK.
Conflicts of interest: none declared.

FNAC can be used as a first-line investigation of a salivary gland


lesion. A needle attached to a 20-ml syringe is inserted into the
lesion applying suction if preferred and several passes made with
a forwardebackward movement. A core biopsy offers improved
diagnostic accuracy, utilizing a wider gauge needle, but is more

James OHara FRCS is a Consultant Otolaryngologist Head and Neck


Surgeon at Sunderland Royal Hospital, Sunderland, UK. Conflicts of
interest: none declared.

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Sialendoscopy
Sialendoscopy is the use of endoscopes in the diagnosis and
treatment of ductal pathology. It is most applicable to patients
who have symptoms of salivary gland swelling on eating;
indicative of a stone or stenosis.2 Interventional sialendoscopes
incorporate a working channel through which baskets, guide
wires, laser fibres and balloons can be passed.

Non-neoplastic disease
Inflammatory conditions
Acute viral inflammation: the commonest cause of acute viral
parotitis is mumps; caused by the paromyxovirus. Patients present with malaise, fever, anorexia and acute bilateral parotid
enlargement. Parotitis may be unilateral and can present with
swelling in the submandibular and sublingual glands. Systemic
complications include meningitis, encephalitis, hepatitis, carditis, orchitis and hearing loss. Treatment is supportive.
Acute suppurative sialadenitis: bacterial infections are uncommon and most frequently occur in the parotid gland. The
most common presentation is of unilateral parotid enlargement
with cellulitis, in a dehydrated elderly patient. Pus is often
demonstrable at the duct orifice. The commonest causative agent
is Staphylococcus aureus. Treatment is broad-spectrum intravenous antibiotics and rehydration. Occasionally, abscess formation can occur requiring incision and drainage.
Chronic inflammatory conditions
Infective:
Mycobacterium tuberculosis (TB) of the salivary glands is
relatively rare in the UK. It may mimic a malignant neoplasm,
with enlargement and pain, most commonly in the parotid gland
due to infection within the peri-parotid lymph nodes. A chest
radiograph may confirm coexistent pulmonary TB. Definitive
diagnosis can be made with aspiration or formal incision
and drainage of pus, allowing identification of acid-fast bacilli
on microscopy and culture. Treatment is anti-tuberculous
chemotherapy.
Atypical tuberculosis is now an increasingly common condition affecting children between the ages of 2 and 5.3 Mycobacterium avium intracellulare is the commonest cause and may
be transmitted through contact with soil. Painless lesions over
either the parotid or submandibular glands occur. The patient is
otherwise well. Whilst combination antibiotics are favoured
some paediatric surgeons advocate excision because untreated it
can discharge as a sinus on a chronic basis, before burning out
leaving scarring of the overlying skin.
Cat scratch disease is a granulomatous disease affecting the
periglandular lymph nodes in the parotid and submandibular
regions. It is caused by the Gram-negative bacterium Bartonella
henselae transmitted through a bite or scratch from a domestic
cat. Serum immunoglobulin G (IgG) and IgM will confirm the
diagnosis. Treatment is supportive, but occasionally surgery
may be considered for non-regressing enlarged, tender
lymphadenopathy.4
Actinomycosis e the Gram-positive anaerobe Actinomyces
israelii may cause painless hard masses in the neck overlying the
salivary glands. Necrosis and multiple sinus tracts often occur

Figure 1 Fine needle aspiration cytology can be performed simultaneously


with diagnostic ultrasound for the parotid and submandibular glands.

Figure 2 T2-weighted, axial MRI scan demonstrating a pleomorphic adenoma of the right parotid gland.

painful for patients and has a higher risk of haematoma. The role
of cytology is to aid clinical distinction between neoplastic and
non-neoplastic disease of the salivary gland and also between
benign and malignant salivary neoplasms.

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Salivary gland cysts:


Retention cysts occur after duct obstruction and can occur
with sialadenitis or sialadenosis. Similar cysts can occur in
Sjogrens syndrome and HIV.
Mucoceles mucus-containing mucosal swellings, mainly
located in the lower lip from a minor salivary gland.
Ranula e a type of extravasation mucocele, these arise from
the sublingual gland and are termed plunging if they penetrate
through the mylohyoid muscle into the neck.

within the lesions. Treatment is surgical debridement or excision


with long-term antibiotics.
Human immunodeficiency virus (HIV) e Generalized parotid gland enlargement and xerostomia may be a presenting
feature of undiagnosed HIV infection. Lymphoepithelial cysts
may occur, commonly bilaterally in the parotid. Conservative
management of HIV-associated cysts is advocated.
Non-infective:
Sialolithiasis e the submandibular gland is most
commonly affected (60e70%) by calculi. The presentation is a
swollen gland on eating e meal-time syndrome. This may
subside after hours, only to return, alternatively recurrent
acute infections of the gland occur, each one causing further
scarring, resulting in a chronically stenotic duct and fibrosed
gland. Acute intermittent episodes can be managed with conservative measures (warm compress, massage, foods that
stimulate saliva) and antibiotics for infection. Small calculi
may pass spontaneously. Chronic symptoms should be investigated with ultrasound, sialography or diagnostic sialendoscopy to determine stone location and ductal patency.
Interventional sialendoscopy,5 has reduced the morbidity of
surgery for sialolithiasis. Where these measures fail or are
unavailable, salivary gland excision may be offered.
gren syndrome (SS) e it is the most common autoimSjo
mune disease affecting the salivary glands. It may be primary or
secondary to another connective tissue disorder. It commonly
affects women between 40 and 60 years. Symptoms are dry,
gritty eyes and a dry mouth. Patients may be referred with
recurrent salivary gland swelling, usually the parotids. Diagnosis is made through a combination of clinical features, histology of minor salivary gland (a sublabial biopsy),
measurement of salivary flow and autoantibodies (anti-Ro and
anti-La).6 Treatment consists of saliva and tear replacement
substitutes. Systemic therapy involves immunomodulatory
medications, or steroids for more severe SS symptoms. Patients
are at a higher risk of developing mucosa-associated lymphoid
tissue (MALT) lymphoma.
Sarcoidosis is an idiopathic multisystem inflammatory
disease characterized by non-caseating granulomata,
commonly affecting young adults. Any organ can be involved,
the salivary glands, usually the parotids, are involved in 10
e30% of patients. Non-tender, diffuse swelling is the usual
presentation. Imaging may show multiple non-cavitating
masses. FNAC is useful in excluding malignancy. The diagnosis may be suggested via a chest radiograph, demonstrating
mediastinal lymphadenopathy with or without pulmonary infiltrates, in association with a raised serum angiotensinconverting enzyme level. Ultimately a tissue diagnosis of
non-caseating granulomata is required. Treatment most
frequently consists of systemic steroids or steroid-sparing
immunosuppressants.

Benign epithelial neoplasms


The World Health Organization (WHO) in 200510 classified
benign tumours into 13 subtypes (Table 1). Because of the
epithelial and myoepithelial tissue components of salivary
glands, the tumours are a heterogeneous group which can be
defined according to their dominant tissue type or can be mixed.

Non-inflammatory conditions
Sialadenosis/sialosis is a non-inflammatory, non-neoplastic,
non-painful, bilateral swelling of the major salivary glands and is
usually most clinically apparent in the parotid glands. There are a
number of factors associated with this condition including drugs,
endocrine disorders and nutritional disorders.

Other benign epithelial neoplasms


The remaining benign epithelial tumours make up about 15% of
all tumours. Their diagnosis depends on expert cyto- and histopathologists to determine benign from malignant processes.
The final diagnosis of a salivary gland neoplasm can often only
be made on definitive histology, one of the main reasons for
performing surgery on clinically benign disease.

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Neoplastic disease
Salivary gland tumours represent 2e4% of all head and neck
neoplasms. They are divided into benign and malignant neoplasms and can be epithelial or non-epithelial in origin. Most
(70%) salivary gland tumours are found in the parotid gland with
8% in the submandibular glands and 22% in the minor glands.7
In one study 25% of parotid neoplasms were malignant, 43% of
submandibular neoplasms and 82% of minor salivary gland
neoplasms.8
Environmental and genetic factors have been proposed in the
aetiology of salivary gland neoplasms. The strongest link seems
to be radiation exposure, highlighted in the Japanese atomic
bomb survivors studies showing a higher incidence in the
exposed population.9 Smoking has been implicated in the
development of Warthins tumours.

Pleomorphic adenoma (PA): it is the most common tumour of


the salivary glands. It is most frequently found in the parotid
gland. Tumours are slow growing painless masses. PAs are of
mixed origin and the epithelial, myoepithelial and mesenchymal
components demonstrate huge cell variation, architecture and
morphology (Figure 3). Surgical excision is the preferred management for these tumours as they have a small but definite
potential for malignant transformation.
Warthin tumour: also known as adenolymphoma, Warthin tumours are the second most common benign neoplasm. Warthin
tumours are cystic and found exclusively in the parotid glands.
Ten percent are bilateral and there is an association with
smoking. Recent evidence questions whether Warthin tumours
are truly parotid neoplasms, or rather are a disease of the periparotid lymph nodes.11 Where possible, the management of
these tumours is surgical.

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Benign non-epithelial neoplasms


Haemangiomas: most commonly affect the parotid gland in
children, the tumours have a characteristic rapid growth phase in
the first 6 months. The diagnosis is entirely clinical and reassurance can be provided that natural resolution will ensue between 1 and 2 years.

World Health Organization classification of epithelial


salivary gland neoplasms10
Benign epithelial
neoplasms

Malignant epithelial neoplasms

Pleomorphic adenoma
Warthin tumour
Myoepithelioma
Basal cell adenoma

Acinic cell carcinoma


Mucoepidermoid carcinoma
Adenoid cystic carcinoma
Polymorphous low-grade
adenocarcinoma
Epithelialemyoepithelial carcinoma
Clear cell carcinoma not otherwise
specified
Basal cell adenocarcinoma
Sebaceous carcinoma
Sebaceous lymphadenocarcinoma
Cystadenocarcinoma
Low-grade cribriform
cystadenocarcinoma
Mucinous adenocarcinoma
Oncocytic carcinoma
Salivary duct carcinoma

Oncocytoma
Canalicular adenoma
Sebaceous adenoma
Lymphadenoma
Sebaceous
Non-sebaceous
Ductal papillomas
Inverted papillomas
Intra-ductal papilloma
Sialadenoma
papilliferum
Cystadenoma

Vascular malformations: these usually present at birth and may


require surgical excision from the major salivary glands.
Lipomas may affect the major salivary glands.
Malignant epithelial neoplasms
The most recent WHO classification10 of malignant salivary
gland neoplasm includes 24 subtypes (Table 1). The majority, 60
e70%, of patients will have either mucoepidermoid carcinoma,
adenoid cystic carcinoma, acinic cell carcinoma or polymorphous low-grade adenocarcinoma. Clinical signs that suggest
a malignant tumour in the salivary gland include, pain, facial or
hypoglossal nerve involvement, a sudden increase in size of a
pre-existing salivary gland tumour and associated cervical
lymphadenopathy.
Mucoepidermoid carcinoma (MEC): it is the most common
malignant neoplasm of the salivary glands. Half of these tumours
present in the major glands, frequently in the parotid gland
(45%). The majority of tumours are low or intermediate grade,
are treated surgically and have a good prognosis but high-grade
tumours have an increased metastatic potential.

Adenocarcinoma, not otherwise specified


Myoepithelial carcinoma
Carcinoma ex-pleomorphic adenoma
Carcinosarcoma
Metastasizing pleomorphic adenoma
Squamous cell carcinoma
Small cell carcinoma
Large cell carcinoma
Lymphoepithelial carcinoma
Sialoblastoma

Adenoid cystic carcinoma: it makes up 10% of malignant salivary gland tumours overall but 30% of minor salivary gland
tumours. The tumours have a predilection for peri-neural spread
and may present with a nerve palsy. Despite local control with
surgery and radiotherapy, 80e90% of patients die of the disease
after 10e15 years due to metastases to the lungs, bone, brain and
liver.

Table 1

Acinic cell carcinoma: 80% of acinic cell carcinomas occur in


the parotid gland. Presentation is a slow-growing mass, occasionally with pain and facial nerve palsy and may be bilateral.
Acinic cell carcinomas tend to metastasize to the cervical lymph
nodes.
Carcinoma ex-pleomorphic adenoma: these tumours are those
in which a new malignancy has arisen in a previous PA.
They represent 12% of malignant salivary gland tumours and
frequently present as a long-standing mass that has recently
increased in size. The treatment of choice is wide local surgical
excision with neck dissection followed by postoperative
radiotherapy.
Metastatic disease to the major salivary glands: the parotid
gland is the gland most frequently involved by metastatic disease
usually by cutaneous squamous cell carcinoma of the head and
neck. Whilst this is relatively rare in the northern hemisphere, it
is the commonest parotid malignancy in Australia and New
Zealand. The parotid gland has a lymphatic network which
drains the temple and cheek regions; common sites for skin

Figure 3 A pleomorphic adenoma composed of ductal structures that


contain mucus secretions (right of field), along with dispersed
reticular networks of cells embedded in a myxoid matrix (left of
field).

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 the tympanomastoid suture line lies between the tympanic ring of bone and the mastoid. This leads to the
stylomastoid foramen
 posterior belly of digastric; the nerve will lie 1 cm deep
to this.
 Once the main trunk of the nerve is identified an artery clip
is inserted on top of the nerve, opened so the nerve can be
seen and the parotid tissue divided superficial to the nerve.
 For an inferiorly placed tumour one of the middle branches
of the nerve can be followed out to the periphery. The
lower branches are then followed out one by one from
superior to inferior so the tumour and gland are brought
inferiorly before being completely excised. For a larger
tumour, all the branches are followed out to the periphery
one by one, often starting superiorly and going inferiorly in
a similar manner (Figure 4).

cancer. Metastases from infraclavicular primary sites are rare,


with the literature limited to case reports from the lung, breast
and kidney.

Surgery principles
Parotid surgery
The commonest indication is for neoplastic disease. The term
superficial parotidectomy implies removal of all parotid tissue
lateral to the facial nerve (Figure 4). In reality, for benign neoplasms, all that is required is removal of the tumour with a cuff of
normal tissue surrounding it. It is widely accepted that a simple
lumpectomy or enucleation of benign neoplasms results in an
unacceptably high recurrence rate. Frequently, a benign tumour
may extend between branches of the nerve therefore entering the
deep lobe. Therefore, the preferred terminology is a partial superficial parotidectomy / deep lobe dissection.12
The key steps of the operation:
 Traditional modified Blairs incision; lying in a skin crease
anterior to the tragus of the pinna, curving under the ear
lobe and then passing into a skin crease at least two finger
breadths below the mandible. The use of a rhytidectomy
(face lift) incision is becoming more popular for improved
cosmesis.
 Facial nerve monitor electrodes inserted.
 Skin flap is raised to the anterior border of the gland.
 Dissection proceeds down to the sternocleidomastoid
muscle identifying the greater auricular nerve.
 Delineation of the anterior border of the sternocleidomastoid allowing dissection onto the posterior belly of digastric
muscle.
 Identification of facial nerve e three landmarks:
 tragal pointer (least reliable) e part of the cartilage of
the external auditory canal. The nerve lies 1 cm deep and
inferior to this

Submandibular gland surgery


Unlike parotid surgery this is more commonly performed for
inflammatory conditions of the gland than neoplasms. Dissection
involves protection of two nerves, the mandibular and lingual
nerves. The mandibular need not be dissected for inflammatory
pathology but must be formally identified and protected, where
appropriate to do so, for neoplastic disease. In order to excise the
gland, the facial vessels must be ligated. To release the gland, the
small parasympathetic nerve branch must be divided where it
comes off the lingual nerve. It is unusual to encounter the hypoglossal nerve. The submandibular duct must be ligated to
avoid contamination from the oral cavity with the wound.

Surgical complications
Intraoperative
Accidental division of the facial nerve should be repaired at the
time with a tension free end-to-end anastomosis using the
operating microscope.
Rupture of the tumour capsule can be repaired with a suture,
where appropriate. If tumour is spilt, all macroscopic tumour
should be removed. Whether to irrigate the tumour bed is
debated. If irrigation is performed it should be with water;
osmosis will rupture the tumour cells.
Postoperative
Facial nerve palsy may be temporary or permanent, partial or
complete. The risk varies with the extent of the surgery, experience of the surgeon, pathology and with recurrent disease.
Three-quarters of temporary nerve palsies resolve by 3 months.
Freys syndrome is sweating, erythema or warmth over the
parotid bed area whilst eating. It is thought to be due to parasympathetic nerve fibres from the auriculotemporal nerve reanastomosing with sweat glands following parotidectomy. A

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Figure 4 Completing a superficial parotidectomy. This shows the correct


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