Chronic sclerosing sialadenitis

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Chronic sclerosing sialadenitis
Specialty Dentistry

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. [1]

Contents

Involvement of the submandibular glands is also known as Küttner's tumor, named after Hermann Küttner (18701932), a German Oral and Maxillofacial Surgeon, who reported four cases of submandibular gland lesions for the first time in 1896. [2]

Presentation

The inflammatory lesions in Küttner's tumor may occur on one side (unilateral) or both sides (bilateral), predominantly involving the submandibular gland, [3] but is also known to occur in other major and minor salivary glands, [4] including the parotid gland. [5] [6] 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. [7] 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. [8] 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.

Histological features

The histopathological features that characterize Küttner's tumor [9] include:

Pathogenesis

The cause and pathogenesis of this chronic condition are not very well understood. Several factors have been postulated:

This chronic condition is primarily observed in adult (40–70 years) patients. However, Küttner's tumor, with prominent immunopathological features, has been described in an 11-year-old boy in Brazil in 2012. [19]

Diagnosis

Given the difficulties of a definitive pre-operative diagnosis, the clinical entity of Küttner's tumor has so far remained significantly under-reported and under-recognized. In recent times, armed with a better understanding of the occurrences and observable features of this condition, surgeons are increasingly depending upon pre-operative ultrasonography along with Fine-needle aspiration cytological (FNAC) examinations to make an accurate presumptive diagnosis, [20] and according to one estimate, about 44% of patients undergoing submandibular resection are found to have this condition. [20] In the ultrasonogram, Küttner's tumor is characterized by a diffuse, heterogeneous zone of echo-shadows. [21] The FNAC finds cells greatly reduced in number (called 'paucicellularity') along with scattered tubular ducts against a backdrop of lymphoplasmacytic infiltration and fibrous depositions. [21] There may be a reduced but moderate number of cells and ducts enveloped in fibrous sheaths, as well as fibrous proliferation of the gland's septa. [22] The cytologic findings by themselves may not be specific, and the diagnosis requires adjunct consideration of both the ultrasonogram and clinical presentation. Application of magnetic resonance imaging (MRI) has been tried to non-invasively examine the morphological variations in Küttner's tumor and differentiate them from those seen in malignant tumors; [23] while MRI findings of the affected tissue and the pattern of cellular infiltration may offer some diagnostic clues for this condition, so far the results have been inconclusive. [23]

Existing treatment

Standard, and most effective, therapy to date is glandular sialadenectomy, which is associated with fairly low operative morbidity; however, in recent times, the administration of steroid (which can shrink the inflammatory lesion and is known to reduce serum IgG4 values) has been considered favorably, [16] [17] and may be useful in younger patients or those who refuse surgery.

See also

Related Research Articles

<span class="mw-page-title-main">Salivary gland</span> Exocrine glands that produce saliva through a system of ducts

The salivary glands in many vertebrates including mammals are exocrine glands that produce saliva through a system of ducts. Humans have three paired major salivary glands, as well as hundreds of minor salivary glands. Salivary glands can be classified as serous, mucous, or seromucous (mixed).

<span class="mw-page-title-main">Parotid gland</span> Major salivary gland in many animals

The parotid gland is a major salivary gland in many animals. In humans, the two parotid glands are present on either side of the mouth and in front of both ears. They are the largest of the salivary glands. Each parotid is wrapped around the mandibular ramus, and secretes serous saliva through the parotid duct into the mouth, to facilitate mastication and swallowing and to begin the digestion of starches. There are also two other types of salivary glands; they are submandibular and sublingual glands. Sometimes accessory parotid glands are found close to the main parotid glands.

<span class="mw-page-title-main">Submandibular gland</span> Human salivary gland

The paired submandibular glands are major salivary glands located beneath the floor of the mouth. In adult humans, they each weigh about 15 grams and contribute some 60–67% of unstimulated saliva secretion; on stimulation their contribution decreases in proportion as parotid gland secretion rises to 50%. The average length of the normal adult human submandibular salivary gland is approximately 27 mm, while the average width is approximately 14.3 mm.

<span class="mw-page-title-main">Parotitis</span> Medical condition

Parotitis is an inflammation of one or both parotid glands, the major salivary glands located on either side of the face, in humans. The parotid gland is the salivary gland most commonly affected by inflammation.

<span class="mw-page-title-main">Warthin's tumor</span> Medical condition

Warthin's tumor, also known as papillary cystadenoma lymphomatosum, is a benign cystic tumor of the salivary glands containing abundant lymphocytes and germinal centers. It is named for pathologist Aldred Scott Warthin, who described two cases in 1929.

<span class="mw-page-title-main">Pleomorphic adenoma</span> Medical condition

Pleomorphic adenoma is a common benign salivary gland neoplasm characterised by neoplastic proliferation of epithelial (ductal) cells along with myoepithelial components, having a malignant potentiality. It is the most common type of salivary gland tumor and the most common tumor of the parotid gland. It derives its name from the architectural Pleomorphism seen by light microscopy. It is also known as "Mixed tumor, salivary gland type", which refers to its dual origin from epithelial and myoepithelial elements as opposed to its pleomorphic appearance.

<span class="mw-page-title-main">Benign lymphoepithelial lesion</span> Medical condition

Benign lymphoepithelial lesion or Mikulicz' disease is a type of benign enlargement of the parotid and/or lacrimal glands. This pathologic state is sometimes, but not always, associated with Sjögren's syndrome.

An oral medicine or stomatology doctor/dentist has received additional specialized training and experience in the diagnosis and management of oral mucosal abnormalities including oral cancer, salivary gland disorders, temporomandibular disorders and facial pain, taste and smell disorders; and recognition of the oral manifestations of systemic and infectious diseases. It lies at the interface between medicine and dentistry. An oral medicine doctor is trained to diagnose and manage patients with disorders of the orofacial region.

<span class="mw-page-title-main">Mucoepidermoid carcinoma</span> Medical condition

Mucoepidermoid carcinoma (MEC) is the most common type of minor salivary gland malignancy in adults. Mucoepidermoid carcinoma can also be found in other organs, such as bronchi, lacrimal sac, and thyroid gland.

<span class="mw-page-title-main">Sialadenitis</span> Medical condition

Sialadenitis (sialoadenitis) is inflammation of salivary glands, usually the major ones, the most common being the parotid gland, followed by submandibular and sublingual glands. It should not be confused with sialadenosis (sialosis) which is a non-inflammatory enlargement of the major salivary glands.

<span class="mw-page-title-main">Sialolithiasis</span> Medical condition

Sialolithiasis is a crystallopathy where a calcified mass or sialolith forms within a salivary gland, usually in the duct of the submandibular gland. Less commonly the parotid gland or rarely the sublingual gland or a minor salivary gland may develop salivary stones.

<span class="mw-page-title-main">Acinic cell carcinoma</span> Medical condition

Acinic cell carcinoma is a malignant tumor representing 2% of all salivary tumors. 90% of the time found in the parotid gland, 10% intraorally on buccal mucosa or palate. The disease presents as a slow growing mass, associated with pain or tenderness in 50% of the cases. Often appears pseudoencapsulated.

<span class="mw-page-title-main">Stafne defect</span> Mandibular depression

The Stafne defect is a depression of the mandible, most commonly located on the lingual surface. The Stafne defect is thought to be a normal anatomical variant, as the depression is created by ectopic salivary gland tissue associated with the submandibular gland and does not represent a pathologic lesion as such. This cavity is commonly observed on panoramic radiograph.

<span class="mw-page-title-main">Salivary gland tumour</span> Medical condition

Salivary gland tumours, also known as mucous gland adenomas or neoplasms, are tumours that form in the tissues of salivary glands. The salivary glands are classified as major or minor. The major salivary glands consist of the parotid, submandibular, and sublingual glands. The minor salivary glands consist of 800 to 1000 small mucus-secreting glands located throughout the lining of the oral cavity. Patients with these types of tumours may be asymptomatic.

Sialoendoscopy is a minimally invasive technique that allows for salivary gland surgery for the safe and effective treatment of obstructive salivary gland disorders and other conditions of the salivary glands. During sialoendoscopy a small endoscope is placed into the salivary glands through the salivary ducts that empty into the mouth. The procedure is not exclusively diagnostic, but is interventional; thus, it can be used for the extraction of salivary stones, salivary duct lavage, dilatation of stenotic segments, or instillation of various medications such as corticosteroids or antibiotics. Thus, sialoendoscopy is an efficient yet simple mode of treatment for major salivary gland obstructions, strictures and sialoliths. Depending on the obstruction, sialoendoscopy can be conducted under local anesthesia in an outpatient office or in the operating room under general anesthesia.

<span class="mw-page-title-main">IgG4-related disease</span> Medical condition

IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis (scarring) and a usually prompt response to oral steroids. In approximately 51–70% of people with this disease, serum IgG4 concentrations are elevated during an acute phase.

<span class="mw-page-title-main">Epithelial-myoepithelial carcinoma</span> Medical condition

Epithelial-myoepithelial carcinoma (EMCa) is a rare malignant tumour that typically arises in a salivary gland and consists of both an epithelial and myoepithelial component. They are predominantly found in the parotid gland and represent approximately 1% of salivary gland tumours.

A sialoblastoma is a low-grade salivary gland neoplasm that recapitulates primitive salivary gland anlage. It has previously been referred to as congenital basal cell adenoma, embryoma, or basaloid adenocarcinoma. It is an extremely rare tumor, with less than 100 cases reported worldwide.

<span class="mw-page-title-main">Salivary gland disease</span> Medical condition

Salivary gland diseases (SGDs) are multiple and varied in cause. There are three paired major salivary glands in humans: the parotid glands, the submandibular glands, and the sublingual glands. There are also about 800–1,000 minor salivary glands in the mucosa of the mouth. The parotid glands are in front of the ears, one on side, and secrete mostly serous saliva, via the parotid ducts, into the mouth, usually opening roughly opposite the second upper molars. The submandibular gland is medial to the angle of the mandible, and it drains its mixture of serous and mucous saliva via the submandibular duct into the mouth, usually opening in a punctum in the floor of mouth. The sublingual gland is below the tongue, on 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.

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Sclerosing polycystic adenosis is a rare salivary gland tumor first described in 1996 by Dr. Brion Smith. The major salivary glands, specifically the parotid gland and the submandibular gland, are affected most commonly. Patients usually come to clinical attention with a mass or swelling in their salivary glands in the 5th decade of life, with females affected much more commonly than males. Nearly all of the cases reported so far have a benign behavior, although there is a single case that has had an associated malignant transformation.

References

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