SALIVARY GLAND TUMOURS

Presenter : Dr Vamsivardhan P

Guide: Dr Ravishankar NProfessor Dept of SurgeryJss medical collegeMysuru

AETIOLOGY Genetic

loss of alleles of chromosomes in 12q, 8q, 17q. Infective

Mumps Epstein-Barr virus chronic sialadenitis: Recurrent inflammation can cause duct

dysplasia and carcinoma. Radiation

More common in survivors of atomic bomb explosion Smoking

Adenolymphoma of Warthin’s shows 40% risk in smokers Sex

Benign tumours and many malignancies are common in females Warthin’s and some malignancies are common in males.

Environment and diet Deficiency of vitamin A Industrial agents like nickel, cadmium, hair dyes, silica,

preservatives

Salivary gland tumoursEpithelial

Non Epithelial

Malignant Lymphomas

Lymphoepithelial Tumours

Secondaries

WHO classification

Epithelial tumours

Adenomas Carcinomas

Pleomorphic adenoma

Monomorphic adenomas

• Adenolymphoma (WARTHINS TUMOUR)

• Oncocytoma

• Basal cell Adenoma

Carcinoma

Low grade High grade

• Acinic cell carcinoma• Adenoid cystic

carcinoma• Low-grade

mucoepidermoid carcinoma

• Adenocarcinoma• Squamous cell carcinoma• High-grade

mucoepidermoid carcinoma

Non Epithelial

Haemangioma Lymphangioma Neurofibromas & Neurilemommas

• Common in infants• Spontaneous regression• MC benign salivary gland tumour in children

Malignant lymphomas Common in parotids. Common with

HIV Sjögren’s syndrome

Secondaries Secondary tumours from

head and neck region bronchus and skin

Lymphoepithelial tumours

Benign

Godwin’s tumour

(BLEL) Benign lymphoepithelial lesion

associated with Mikulicz’s disease or Sjögren’s syndrome

Malignant

(ESKIMOMA)Rare tumour occurs in

parotid and submandibular glands

INCIDENCE 80% per cent salivary neoplasms are in the

parotids of which 80% are benign; 80% of these are pleomorphic adenomas.

50% per cent of salivary tumours are in the submandibular salivary gland, of which 50% are benign. 95% of these are pleomorphic adenomas.

10% percent of salivary neoplasms are in the minor salivary glands : Of these only 10% are benign.

PLEOMORPHIC ADENOMA (MIXED SALIVARY TUMOUR) Commonest of the salivary gland tumour in

adult. More common in parotids (80%). 10% submandibular salivary gland 0.5% sublingual salivary gland.

Grossly it contains cartilages, cystic spaces, solid tissues.

Microscopically it is biphasic in nature with epithelial and stromal components

Common in females (3:1).

Common in 4th and 5th decade

Unilateral.

Present as a single painless, smooth, firm lobulated, mobile swelling in front of the parotid with positive curtain sing

Obliteration of retromandibular groove is common.

Capsulated, tumour may have pseudopods and may extend beyond the main limit of the tumour

Involves superficial lobe superficial and deep lobe together.

Deep lobe involvement Presents as swelling in the lateral wall of the

pharynx, soft palate and posterior pillar

Deep lobe is involvment

Commonly located in the lateral wall of pharynx, posterior pillar and over the soft palate

No visible swelling in the preauricular region.

Dumbbell tumour

passes through Patey’s stylomandibular tunnel pushing tonsils, pharynx, soft palate often without any visible swelling

Bidigital palpation of parotid is significant in such occasion with one finger inside mouth.

Facial nerve is not involved.

CLINICAL FEATURES Swelling pain ulceration dysphagia Raised ear lobule cannot be moved above the

zygomatic bone curtain sign Deviation of uvula and pharyngeal wall towards

midline in case of deep lobe tumour If Malignant :

Facial nerve masseter skin lymph node bone

Complications Recurrence : 5-50% Malignancy

3-5% in early tumours 10% in long duration (15 or more years) tumours

Investigations FNAC CT scan (deep lobe, local extension metastasis) MRI

Long-standing pleomorphic adenoma may turn into carcinoma

Features suggesting Carcinoma Recent increase in size

Pain and nodularity Involvement of skin, ulceration Involvement of masseter

Involvement of cervical lymph nodes

Restriction of jaw movements

Involvement of facial nerve : LMN facial nerve palsy

TREATMENT Surgery :first line treatment

only superficial

lobe is involved

superficial parotidectomy

Both the

lobes involved

Total conservative parotidecto

myfacial nerve is retained

ADENOLYMPHOMA (WARTHIN’S TUMOUR)

Papillary Cystadenolymphomatosum

misnomer

Benign tumour that occurs only in parotid usually in the lower pole.

Trapping of jugular lymph sacs in parotid during developmental period.

Only superficial lobe of parotid gland involvement

May also be multicentric.

Smoking (40%/8 times more risk than nonsmokers)

Radiation exposure

CLINICAL FEATURES

Slow growing, smooth, soft, cystic, fluctuant swelling, in the lower pole Bilateral and is nontender. Common in males; 4:1. Common in smokers. Common in old people > 60 years. Incidence is 10% 2nd most common benign tumour. Often bilateral—10%.

INVESTIGATIONS Technetium99 pertechnetate scan

Adenolymphoma produces a “hot spot” diagnostic

FNAC.

Adenolymphoma does not turn into malignancy occasionally it can simultaneously be associated

with pleomorphic adenoma carcinoma lymphoma of parotid.

Treatment Superficial parotidectomy.

ONCOCYTOMA (OXYPHIL ADENOMA) < 1% of salivary tumours. Usually benign, originating from oncocytes

(oxyphilic cells) Causes.

Radiation occupational hazards

Common in parotid Rarely can occur in submandibular salivary

gland. Investigations.

99Tc with hotspots FNAC

BASAL CELL ADENOMA Rare, benign tumour Common in minor salivary glands In major salivary glands it is multicentric Grossly it looks like lymph node Microscopy : isomorphic basaloid cells with

solid/ tubular/membranous pattern

Canalicular adenoma variant with bilayered ribbons of columnar cells

separated by vascular stroma

MUCOEPIDERMOID TUMOUR

Commonest malignant tumour in parotid

2nd common malignant tumour in submandibular and sublingual salivary glands

Incidence : 9% of salivary tumours

20% of malignant salivary tumours

Occurs both in major as well as minor salivary glands.

Parotid is the commonest site

Palate is the commonest minor salivary gland site (In the palate adenoid cystic carcinoma is common).

Radiation is the commonest etiological factor.

Slowly progressive, often attains a large size and spreads to neck lymph nodes.

Contains malignant epidermoid and mucous secreting cells.

Types Low grade : mucous cells mainly , spreads to

regional nodes.

Intermediate : clear cell variety

High grade : epidermoid cells mainly : spreads to regional nodes and also shows high propensity for distant spread.

Facial nerve involvement is late

CLINICAL FEATURES Swelling in the salivary (parotid or

submandibular) region

Slow increasing in size

Attains a large size, which is hard, nodular, often with involvement of skin and lymph nodes

Common in females (3:1) , slow growing

Pain, skin and facial involvement are not common unless it is high grade

ADENOID CYSTIC CARCINOMA(10% OF SALIVARY TUMOURS)Cylidromatous carcinoma Most common tumour in Sublingual salivary glands

50% of cases occur in minor salivary glands: palate

2nd most common malignant salivary tumour

Rare in parotid (2% of parotid tumours, 15% of malignant parotid tumours

Common in females (3:2)

Common in 5th and 6th decades

Slow growing but highly malignant

Remarkable capacity for recurrence

High affinity for perineural spread

Blood spread can occur to lungs, bones and liver

Lung secondaries remain dormant not a contraindication for surgery of primary

tumour

Treatment of choice

Radical parotidectomy

radical excision of submandibular and sublingual glands with neck nodal dissection and postoperative

RT

Poor prognosis on microscopy Positive margin perineural spread solid type

Lung metastasis will not affect the prognosis.

Local recurrence is common

5-year survival is 70%.

ACINIC CELL TUMOUR Rare, slow growing tumour that occurs almost always

in parotid and is composed of cells alike serous acini.

More common in women

Occurs in adult and elderly

3% of salivary tumours : 90% occurs in parotid.

Can involve facial nerve or neck lymph nodes.

Clinically it is of variable consistency with soft and cystic areas.

low grade malignant tumour

MALIGNANT MIXED TUMOUR (MMT) 10% of salivary malignancy in incidence with

epithelial and mesenchymal elements. Carries worst prognosis.

MMT TypesCarcinoma ex pleomorphic adenoma

Primary malignant mixed tumour

Metastasizing mixed tumours

In situ non-invasive carcinoma in pleomorphic adenoma

Carcinoma ex pleomorphic adenoma: Commonest type

long standing parotid swelling shows rapid change, fixity, facial nerve spread, neck nodal involvement

Transformation is 2% in tumour of 5 years duration; 10% in 15 years tumour

Most aggressive salivary malignancy

Treatment. Radical parotidectomy

Primary malignant mixed tumour:

Also called as carcinosarcoma which arises as de novo

Shows components of both carcinoma and sarcoma with metastatic potential both through lymph nodes and blood.

Metastasizing mixed tumours:

contains structures typical of benign mixed tumour both at original and at metastases sites.

In situ non-invasive carcinoma in pleomorphic adenoma:

No evidence of capsular invasion.

Lesion with

< 8 mm invasion in depth shows 100% of 5-year survival

> 8 mm invasion carries 5-year survival < 50%.

ADENOCARCINOMA OF SALIVARY GLANDS

3% of parotid 10% of submandibular and minor salivary

gland tumours Equal in both sexes Common in children Can be

tubular papillary undifferentiated

20% involve facial nerve clinically.

Undifferentiated type is aggressive.

SQUAMOUS CELL CARCINOMA OF SALIVARY GLANDS Rare in salivary glands.

Parotid is the common site

high grade tumour

Common in men (3:1).

Occurs in 6th or 7th decade

Aggressive nonencapsulated tumour arising from ductal system grows rapidly causing pain, facial palsy, skin fixity , ulceration.

Commonly spreads to neck nodes

Poor prognosis.

Treatment Radical parotidectomy

RT

PAROTID LYMPHOMA Occur from the lymph nodes in the gland or

from parotid parenchyma.

Occur HIV patients lymphoepithelial diseases Sjögren’s syndrome.

Common in elderly.

Confined to parotid gland or may involve other nodes in neck, mediastinum.

Confined to parotid Total parotidectomy with radiotherapy and later

chemotherapy is the treatment.

Multiple lymphnodes are involved chemotherapy is the choice therapy

SUBMANDIBULAR SALIVARY GLAND TUMOURSBenign tumours: Commonly pleomorphic adenomas, are smooth, firm or hard, bidigitally palpable, without involving adjacent muscles or hypoglossal nerve or mandible bone.

Diagnosis FNAC, Orthopantomogram (OPG) and CT scan.

Excision of both superficial and deep lobes of the gland is done.

Malignant tumours of submandibular salivary gland:

hard, nodular

fixed to skin muscles hypoglossal nerve mandible

Diagnosis is by FNAC of primary tumour and of lymph nodes when involved

CT scan

MANAGEMENT

Excision of the gland

Lymph nodes are involved block dissection of neck Classical neck dissection is done

MINOR SALIVARY GLAND TUMOURS 10% of salivary tumours.

common in palate (40%). lip. cheek. sublingual glands.

10% are benign : commonly pleomorphic adenomas.

90% are malignant : commonly adenoid cystic carcinomas.

Present as swelling with ulcer over the summit.

Malignant extends into the palate, maxilla, pterygoids can

occur often with involvement of the lymph node

Differential Diagnosis Squamous cell carcinoma of oral cavity.

Investigations Incision biopsy. CT scan. X-ray maxilla. FNAC of lymph node

MANAGEMENT

Malignancy Wide excision often with palatal excision or

maxillectomy is done Tumour < 1 cm in size :

Excision biopsy is done with 1 cm clearance margin.

Tumour > 1 cm in size : Initially incision biopsy is done and then wide excision is done.

Bony palate is infiltrated that part of palate bone is removed area is reconstructed Reconstruction by dental plates, skin grafting, or

flaps are done.

Lymphnode block dissection of the neck is done if cervical lymphnodes involved.

benign tumours Excision with primary closure is done

SPECIFIC INVESTIGATIONS

FNAC

Confirms possibility Lymphoma/inflammatory masses.

Allows preoperative assesment ofNature of tumourExtent of resection (conservative/radical)Management of facial nerve (high grade

adenoid cystic)Likelihood of neck dissection (high grade).

CT scan Deep lobe involvement Involvement of bone Extension into the base of theskull Relation of tumour to internal carotid artery, styloid process, deep lobe tumour, parapharyngeal space extension Neck nodes are better assessed Bony changes in the foramina, erosions and

sclerotic margins in fissures or canals, enlarged diameter of canal and fissure are the CT features of perineural spread.

MRI

Find out perineural spread

Bone marrow involvement, skull bones, internal architecture and intracranial extensions, recurrent tumours

Replacement of perineural fat with tumour, contrast (gadolinium) enhancement, increased size of the nerve are features of perineural spread

GENERAL FEATURES OF MALIGNANT SALIVARY TUMOURS Fixation

Resorption of adjacent bone

Pain and anaesthesia in the skin and mucosa

Muscle paralysis

Skin involvement and nodularity

Involvement of jaw and masticatory muscle

Nerve involvement

Blood spread when occurs, commonly to lungs

Mandibular branch of 5th cranial nerve involved

TNM Staging of Malignant Salivary Tumours

T: Tumour Tx : Tumour cannot be assessed. T0 : No evidence of primary tumour. T1 : Tumour < 2cm without extraparenchymal spread. T2 : Tumour 2-4 cm, without extraparenchymal extension T3 : Tumour >4 cm. or with extraparenchymal spread. but no facial nerve spread. T4 : T4a :Spread to facial nerve, skin, mandible, ear canal T4b :spread to base of skull, pterygoid plates, encased external carotid artery

N : Lymph node

Nx : Nodes not assessed. N0 : Regional nodes not involved. N1 : Single ipsilateral node < 3 cm. N2a : Single ipsilateral node 3-6 cm. N2b : Multiple ipsilateral nodes < 6 cm. N2c : Bilateral or contralateral nodes < 6 cm. N3 : Single node spread > 6 cm

M—Metastases

M0 : No blood spread. M1 : Blood spread present

STAGING

Stage I T1 N0 M0

Stage II T2 N0 M0

Stage III T3 N0/N1 Mo

Stage IVA T4a N2N1N0 M0

Stage IVB T4b any NM0; any T N3M0

Stage IVC Any T any N M1

SURGICAL MANAGEMENT Indications for surgery

T1, T2, T3 tumours of low grade

Total conservative parotidectomy

T4 tumourshigh grade tumours

SCC

Radical parotidectomy

PAROTIDECTOMYtypes

Superficial parotidectomy

Total conservative parotidectomy

Suprafacial parotidectomy

Radical parotidectomy

Superficial parotidectomy:

Removal of superficial lobe of the parotid (superficial to facial nerve)

Done in case of benign diseases of superficial lobe of the parotid. Ex: Pleomorphic Adenoma

Lazy ‘S’ incision—modifi ed Blair's/Sistrunk's approach and raising the skin flaps

Mobilisation of the gland Flap is reflected in front just up to anterior

margin of the parotid

After identification of sternocleidomastoid great auricular nerve is identified and can be sacrificed

Posterior belly of digastric is identified.

Location of stylomastoid branch of posterior auricular artery is anterior to facial nerve trunk which enters the stylomastoid foramen.

Identification of facial nerve trunk Dissection of the gland off the facial nerve

using bipolar cautery

Removal of parotid—superficial

Distilled water (hypertonic) irrigation to kill spilled tumour cells

Haemostasis and closure with a suction drain

Total conservative parotidectomy Done in benign diseases of parotid involving

either only deep lobe or both superficial and deep lobes

Both lobes are removed

Preservation of facial nerveInitially superficial parotidectomy is done

Facial nerve and its branches are retracted

Deep lobe is removed

Deeper part of the deep lobe is dissected of the stylopharyngeus and stylohyoid muscles by blunt dissection while retracting the deep lobe

Deep lobe can be dissected out superior/inferior/in between nerve branches.

Haemostasis should be achieved using bipolar cautery.

Main trunk of the facial nerve is stimulated with 0.5 mA to confirm the anatomical and physiological integrity of the branches of the facial nerve

RADICAL PAROTIDECTOMY

Removal of Both lobes of parotid Facial nerve Mandibular ramus Masseter muscle Infratemporal fossa dissection Subtotal petrosectomy

Indications for facial nerve sacrifice

Preoperative weakness/paralysis of nerve

Intraoperative evidence of gross invasion even in presence of normal preoperative function

Tumour involvement through facial nerve from superficial to deep lobe

Positive frozen section of nerve stump mastoidectomy and nerve dissection in temporal

bone is done

Facial nerve is reconstructed Greater auricular nerve Sural nerve

All branches except buccal branch are repaired using cable graft

Nerve graft is not a contraindication for future RT

Radical neck dissection

If Lymph nodes are involved, which is confirmed by FNAC is done

Done in N0 with high-grade tumour or T3/T4 tumours

Suprafacial parotidectomy

Done in lower pole parotid tumours where in all branches of the facial nerve need not be dissected

RADIOTHERAPY Given in 3-6 weeks after surgery Dose is 50-70 Gy (1.5- 2.0 Gy in 5-8 weeks)

Indications T3, T4 tumours High grade tumours Perineural spread Adenoid cystic carcinomas Deep lobe tumour Vascular involvement Close clearance margin Multiple neck nodes involvement Recurrent malignant tumours Recurrent pleomorphic adenoma Spillage after surgery for pleomorphic adenoma Residual/refractory tumours/nerve involvement

Preoperative radiotherapy

Given in large tumours to reduce the size and make it better operable, i.e. to down stage the disease

Postoperative radiotherapy

Useful to reduce the chances of relapse

External radio therapy is given

Given in all carcinomas

More useful in Adenoid cystic and Squamous cell carcinomas

COMPLICATIONS OF PAROTIDECTOMY Facial nerve injury Haemorrhage Salivary fistulas

Infection : Flap necrosis is common

Frey’s syndrome

Sialocele Numbness over the face and ear - due to injury to

great auricular nerve Spontaneous recovery may occur in 1½ to 2 years

FREY’S SYNDROME Auriculotemporal SyndromeGustatory Sweating Occurs in 10% of cases. Injury to the auriculotemporal nerve

Inappropriate regeneration of the damaged

parasympathetic autonomic nerve fibers to the overlying skin

post-ganglionic parasympathetic fibres from the

otic ganglion

sympathetic nerves from the

superior cervical

ganglion

united

Auriculotemporal nerve has got two branches.

Auricular branch supplies external acoustic meatus, surface of tympanic membrane, skin of auricle above external acoustic meatus

Temporal branch supplies hairy skin of the temple.

Sweating and hyperaesthesia occurs in this area of skin

Causes

Surgeries or accidental injuries to the parotid.

Surgeries or accidental injuries to temporoman dibular joint

Clinical Features Flushing, sweating, erythema, pain and

hyperaesthesia in the skin over the face innervated by the auriculotemporal nerve, whenever salivation is stimulated (i.e. during mastication).

Starch iodine test Involved skin is painted with iodine and driedDry starch applied over this area will become

blue due to more sweat in the area in Frey’s syndrome

Treatment Initially conservative and reassurance

Most often they recover without any active treatment in 6 months

Antiperspirants : aluminium chloride

Anticholinergics like scopolamine 3%, glycopyrrolate 1%, methyl sulfate, radiation 50 Gy are used

Injection of botulinum toxin to the affected skin

Prevented on table by placing muscle (sternomastoid) or fascial (temporalis) flaps or artificial membranes over parotid bed, under the skin

Jacobsen nerve neurectomy: 10% require surgical division of the tympanic

branch of the glossopharyngeal nerve below the round window of middle ear

MANAGEMENT OF SUBMANDIBULAR GLAND TUMOURS Surgical excision is the goal. Intracapsular submandibular gland excision

Tumour is small and entirely encased within the submandibular gland parenchyma, straightforward is appropriate.

Suprahyoid neck dissection benign tumours that are large and project

beyond the submandibular gland Neck dissection or radical neck dissection is

appropriate malignancy

SUPRAOMOHYOID NECK DISSECTION En bloc removal of cervical lymph node groups I-

III

Posterior limit is the cervical plexus and posterior border of the SCM

Inferior limit is the omohyoid muscle overlying the IJV

EXCISION OF THE SUBMANDIBULAR SALIVARY GLAND Neck extended with chin towards opposite

side Incision is made 2-4 cm below and parallel to

margin of the mandible of 6-8 cm in length

Marginal mandibular nervesubplatysmal plane in neckavoid injury to this nerve

Superficial lobe of the glandexposed fully in digastric triangle between

two bellies of digastric and stylohyoid muscles below

Dissection begins at the lower end of the gland by incising the fascia just above the digastric muscle.

Superficial lobe of the gland is mobilised upwards Hypoglossal nerve at lower part very close to the

digastric tendon which is identified and safeguarded

Mylohyoid muscle is retracted so as to remove the deep portion of the gland which is in front of the hyoglossus and mylohyoid

Submandibular ganglion is often seen just above the gland which can be sacrificed.

Wharton’s duct identification and ligation is done

RADICAL EXCISION OF SUBMANDIBULAR SALIVARY GLAND Done in malignant tumours of the

submandibular salivary glan

Nerves are sacrificed if infiltrated

Marginal mandibular nerve Difficult to retain for oncological clearance

Radical neck lymph node dissection is also usually done

CONCLUSION Salivary gland tumours are usually benign in

an adult It is rare in children but when it occurs, it is

commonly malignant Clinical and FNAC are diagnostic methods CT scan or MRI are often needed Nerve should be preserved in benign lesions Nerve can be sacrificed to achieve clearance

in malignancies