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An "accessory salivary gland" is ectopic salivary gland tissue with a salivary gland duct system. The most common location of accessory salivary gland tissue is an extra major salivary gland in front of the parotid gland. It is typically about 3 cm or less in size, and drains into the parotid duct via a single tributary. Accessory parotid tissue is found in 21-56% of adults. Any disease process which affects the salivary glands, including cancer, may also occur within an accessory salivary gland tissue.
Ectopic salivary gland tissue which is located in sites other than the normal location is variously described as aberrant, accessory, ectopic, heterotopic or salivary gland choristoma.
Congenital disorders of the salivary glands are rare, but may include:
- Aplasia
- Atresia
- Ectopic salivary gland tissue
- Stafne defect - an uncommon condition which some consider to be an anatomic variant rather than a true disease. It is thought to be created by an ectopic portion of salivary gland tissue which causes the bone of the mandible to remodel around the tissue, creating an apparent cyst like radiolucent area on radiographs. Classically, this lesion is discovered as a chance finding, since it causes no symptoms. It appears below the inferior alveolar nerve canal in the posterior region of the mandible.
Salivary gland diseases (SGD) are multiple and varied in cause.
There are 3 paired major salivary glands in humans (the parotid gland, the submandibular gland, and the sublingual gland), as well as about 800-1000 minor salivary glands in the oral mucosa of the mouth. The parotid gland is located in front of the ear, and it secretes its mostly serous saliva via the parotid duct (Stenson duct) into the mouth, usually opening roughly opposite the maxillary second molar. The submandibular gland is located medial to the angle of the mandible, and it drains its mixture of serous and mucous saliva via the submandibular duct (Wharton duct) into the mouth, usually opening in a punctum located in the floor of mouth. The sublingual gland is located below the tongue, in the floor of the mouth. It drains its mostly mucous saliva into the mouth via about 8-20 ducts which open along the plica sublingualis (a fold of tissue under the tongue).
The function of the salivary glands is to secrete saliva, which has a lubricating function, which protects the oral mucosa of the mouth during eating and speaking. Saliva also contains digestive enzymes (e.g. salivary amylase) and has antimicrobial action and acts as a buffer. Persons with reduced salivary flow or hyposalivation often suffer from dry mouth or xerostomia, which can result in severe dental caries (tooth decay) as a result of the loss of the protective effects of saliva.
Various examples of disorders affecting the salivary glands are listed below. Some are more common than others, and they are considered according to a surgical sieve, but this list is not exhaustive. Sialadenitis is inflammation of a salivary gland, usually caused by infections, although there are other less common causes of inflammation such as irradiation, allergic reactions or trauma.
Salivary gland aplasia (also termed salivary gland agenesis) is the congenital absence of salivary glands. Usually the term relates to the absence of some or all of the major salivary glands.
It is a rare condition, and most known cases have been in association with syndromes of the ectodermal tissues, particularly the lacrimal apparatus. Example syndromes which have been reported with salivary gland aplasia include hereditary ectodermal dysplasia, mandibulofacial dysostosis and hemifacial microsomia.
The main significance of the condition is a lack of saliva, causing xerostomia (dry mouth), with accompanying susceptibility to dental caries (tooth decay), infections of the mouth, and upper respiratory tract infections (e.g., candidiasis, ascending sialadenitis, laryngitis and pharyngitis). Patients with salivary gland aplasia typically require regular application of topical fluoride to prevent tooth decay.
Salivary gland hyperplasia is hyperplasia of the terminal duct of salivary glands.
There are two types:
- Acinar adenomatoid hyperplasia
- Ductal adenomatoid hyperplasia
Sclerosing polycystic adenosis is a rare, reactive inflammatory condition of the salivary glands. It may be mistaken for salivary gland neoplasia. It does not seem to be a fatal disease.
The inflammatory lesions in Küttner's tumor may occur on one side (unilateral) or both sides (bilateral), predominantly involving the submandibular gland, but is also known to occur in other major and minor salivary glands, including the parotid gland.
Overall, salivary gland tumors are relatively rare, with approximately 2.5–3 cases per 100,000 people per year seen in the Western world; however, salivary gland malignancies account for 3–5% of all head and neck cancers. However, salivary tumors show a great deal of morphological diversity, as well as variations in the nature of the lesion (malignant vs. benign): approximately 20% to 25% of parotid tumors, 35% to 40% of submandibular tumors, and more than 90% of sublingual gland tumors are malignant. This situation underscores the diagnostic challenges in respect of Küttner's tumor; despite being benign, this condition mimics the clinical appearance of malignancy in the salivary gland.
The swollen masses of Küttner's tumor are generally painful, and patients are advised surgical resection (known as 'sialadenectomy') of a part or whole of the glandular tissue upon suspicion of possible malignancy. It is only upon post-surgical histopathology of the excised mass that the diagnosis of Küttner's tumor is definitively made.
Signs and symptoms are variable and depend largely upon whether the obstruction of the duct is complete or partial, and how much resultant pressure is created within the gland. The development of infection in the gland also influences the signs and symptoms.
- Pain, which is intermittent, and may suddenly get worse before mealtimes, and then slowly get better (partial obstruction).
- Swelling of the gland, also usually intermittent, often suddenly appearing or increasing before mealtimes, and then slowly going down (partial obstruction).
- Tenderness of the involved gland.
- Palpable hard lump, if the stone is located near the end of the duct. If the stone is near the submandibular duct orifice, the lump may be felt under the tongue.
- Lack of saliva coming from the duct (total obstruction).
- Erythema (redness) of the floor of the mouth (infection).
- Pus discharging from the duct (infection).
- Cervical lymphadenitis (infection).
- Bad Breath.
Rarely, when stones form in the minor salivary glands, there is usually only slight local swelling in the form of a small nodule and tenderness.
A salivary gland fistula (plural "fistulae") is a fistula (i.e. an abnormal, epithelial-lined tract) involving a salivary gland or duct.
Salivary gland fistulae are almost always related to the parotid gland or duct, although the submandibular gland is rarely the origin.
The fistula can communicate with the mouth (usually causing no symptoms), the paranasal sinuses (giving rhinorrhea) or the facial skin (causing saliva to drain onto the skin).
The usual cause is trauma, however salivary fistula can occur as a complication of surgery, or if the duct becomes obstructed with a calculus.
Most parotid fistulae heal by themselves within a few weeks.
Chronic sclerosing sialadenitis is a chronic (long-lasting) inflammatory condition affecting the salivary gland. Relatively rare in occurrence, this condition is benign, but presents as hard, indurated and enlarged masses that are clinically indistinguishable from salivary gland neoplasms or tumors. It is now regarded as a manifestation of IgG4-related disease.
Involvement of the submandibular glands is also known as Küttner's tumor, named after Hermann Küttner (1870–1932), a German Oral and Maxillofacial Surgeon, who reported four cases of submandibular gland lesions for the first time in 1896.
The term is derived from the Greek words "sialon" (saliva) and "lithos" (stone), and the Latin "-iasis" meaning "process" or "morbid condition". A "calculus" (plural "calculi") is a hard, stone-like concretion that forms within an organ or duct inside the body. They are usually made from mineral salts, and other types of calculi include tonsiloliths (tonsil stones) and renal calculi (kidney stones). "Sialolithiasis" refers to the formation of calculi within a salivary gland. If a calculus forms in the duct that drains the saliva from a salivary gland into the mouth, then saliva will be trapped in the gland. This may cause painful swelling and inflammation of the gland. Inflammation of a salivary gland is termed "sialadenitis". Inflammation associated with blockage of the duct is sometimes termed "obstructive sialadenitis". Because saliva is stimulated to flow more with the thought, sight or smell of food, or with chewing, pain and swelling will often get suddenly worse just before and during a meal ("peri-prandial"), and then slowly decrease after eating, this is termed "meal time syndrome". However, calculi are not the only reasons that a salivary gland may become blocked and give rise to the meal time syndrome. Obstructive salivary gland disease, or obstructive sialadenitis, may also occur due to fibromucinous plugs, duct stenosis, foreign bodies, anatomic variations, or malformations of the duct system leading to a mechanical obstruction associated with stasis of saliva in the duct.
Salivary stones may be divided according to which gland they form in. About 85% of stones occur in the submandibular gland, and between 5-10% occur in the parotid gland. In about 0-5% of cases, the sublingual gland or a minor salivary gland is affected. When minor glands are rarely involved, caliculi are more likely in the minor glands of the buccal mucosa and the maxillary labial mucosa. Submandibular stones are further classified as anterior or posterior in relation to an imaginary transverse line drawn between the mandibular first molar teeth. Stones may be radiopaque, i.e. they will show up on conventional radiographs, or radiolucent, where they not be visible on radiographs (although some of their effects on the gland may still be visible). They may also symptomatic or asymptomatic, according to whether they cause any problems or not.
Salivary gland atresia is congenital blockage or absence of the orifice of a major salivary gland duct or part of the duct itself.
It is a very rare condition. The submandibular salivary gland duct is usually involved, having failed to cannulate during embryological development. The condition first becomes apparent in the first few days after birth where a submandibular swelling caused by a retention cyst is noticed.
Salivary gland tumours usually present as a lump or swelling in the affected gland which may or may not have been present for a long time. The lump may be accompanied by symptoms of duct blockage (e.g. xerostomia). Usually, in their early stages it is not possible to distinguish a benign tumour from a malignant one. One of the key differentiating symptoms of a malignant growth is nerve involvement. For example signs of facial nerve damage (e.g facial palsy) are associated with malignant parotid tumours. Facial pain, and paraesthesia are also very often associated with a malignant tumours. Other red flag symptoms which may suggest malignancy and warrant further investigation are fixation of the lump to the overlying skin, ulceration and induration of the mucosa.
Salivary gland hypoplasia is relative underdevelopment of the Salivary glands. Salivary gland hypoplasia tends to produce xerostomia (dry mouth), with all the associated problems this brings.
It is a rare condition, which may occur as a congenital abnormality or result from lack of neuromuscular stimulation.
It may be associated with Melkersson–Rosenthal syndrome, and hereditary ectodermal dysplasia.
It may appear on a CT scan or MRI scan as enhancement and dilation of the duct (sialectasis).
On sialography, it may appear as segments of duct dilation and stenosis. This is sometimes termed the 'sausage link appearance'.
Little is known about the total incidence of salivary gland tumours as most benign tumours go unrecorded in national cancer registries. The majority of salivary tumours are benign (65-70%). Within the parotid gland 75 - 80% of tumours are benign. Around 50% of the tumours found in the submandibular glands are benign. Sublingual gland tumours are very rare but if present, they are most likely to be malignant.
In the United States, salivary gland cancers are uncommon with an incidence rate of 1.7 in 100000 between 2009 and 2013.
Sialodochitis (also termed ductal sialadenitis), is inflammation of the duct system of a salivary gland. This is compared to sialadenitis, which is inflammation of the gland parenchyma.
Sialodochitis may be associated with salivary duct strictures and salivary stones.
It is common in both the parotid glands and submandibular glands.
The treatment is as for sialadenitis.
Sebaceous lymphadenoma is a tissue diagnosis, e.g. salivary gland biopsy.
It may be confused with a number of benign and malignant neoplasms, including Warthin tumour, mucoepidermoid carcinoma and sebaceous lymphadenocarcinoma.
Studies done on cadavers claim persistent thyroglossal ducts can be completely asymptomatic and found in 7% of the human adult population. However, the continued presence of the duct can often lead to complications due to infections and fluid buildup. The glands in the mucosa of the duct will continue their secretions until the fluid forms a cyst or exit the duct via the opening in the foramen cecum. Local infections, such as colds, tonsillitis, or inflammation of the lymph nodes in the area can also lead to the accumulation of fluid within the duct. Even if the cyst forms as secondary to another infection and improved after antibiotics, it will often reoccur and require treatment.
Three-fourths of abnormalities within a persistent thyroglossal duct involve the formation of a cyst. If a persistent thyroglossal duct becomes fluid filled it will form a thyroglossal duct cyst, which accounts for 70% of congenital neck masses and is the most likely diagnosis if the mass is along the midline of the neck. These cysts are often diagnosed in children under the age of ten and have no particular gender prevalence. The cysts are normally asymptomatic at this age and are noticed because of the swelling that will move if the patient swallows. Over 80% of these cysts are located at or below the hyoid bone.
Very rarely, the persistent duct can become cancerous, called thyroglossal duct carcinoma. In the case of thyroglossal duct carcinoma, the cancerous cells are ectopic thyroid tissue that has been deposited along the thyroglossal duct and will present as a papillary carcinoma. However, the cells are less likely to metastasize in the cyst, than if they were present in the actual thyroid gland.
The other fourth of abnormalities presents as draining abscess. This is from an infection that will rupture through the skin to allow for adequate drainage of the infected area.
The treatment is simple excision and exclusion of a malignant neoplasm.
A ranula usually presents as a translucent blue, dome-shaped, swelling in the tissues of the floor of the mouth. If the lesion is deeper, then there is a greater thickness of tissue separating from the oral cavity and the blue translucent appearance may not be a feature. A ranula can develop into a large lesion many centimeters in diameter, with resultant elevation of the tongue and possibly interfering with swallowing (dysphagia). The swelling is not fixed, may not show blanching and is non-painful unless it becomes secondarily infected. The usual location is usually lateral to the midline, which may be used to help distinguish it from a midline dermoid cyst. A cervical ranula presents as a swelling in the neck, with or without a swelling in the mouth. In common with other mucoceles, ranulas may rupture and then cause recurrent swelling. Ranulas may be asymptomatic, although they can fluctuate rapidly in size, shrinking and swelling, making them hard to detect.
A persistent thyroglossal duct is a usually benign medical condition in which the thyroglossal duct, a structure usually only found during embryonic development, fails to atrophy. The duct persists as a midline structure forming an open connection between the back of the tongue and the thyroid gland.This opening can lead to fluid accumulation and infection, which necessitate the removal of the duct.
The typical presentation is a rapidly growing mass with associated pain. This may be seen in association with neck lymph node swelling (cervical lymphadenopathy), due to metastases, and facial nerve paralysis.
The first signs of a parathyroid adenoma and the resulting primary hyperparathyroidism can include bone fractures and urinary calculi such as kidney stones.
Oftentimes parathyroid adenoma is not diagnosed until found on standard blood-tests that reveal high calcium content in the blood, it can appear in urine tests as well. Patients may not be experiencing any noticeable symptoms but could be producing excessive amounts of calcium and eventually experience problems later in life if untreated. However, patients can experience common symptoms that can range from joint, muscle, and abdominal pain to slight discomfort. Additionally patients might be experiencing feelings of depression due to the hormonal imbalance. Constipation and exhaustion can also be experienced as a result of the irregularity in the bloodstream. There is also a potential that the kidneys could be damaged with the excess of calcium in the blood.