Presentation and Epidemiology
- Inflammatory lesions in Küttners tumor can occur unilaterally or bilaterally.
- Predominantly involves the submandibular gland but can occur in other salivary glands.
- Salivary gland tumors are relatively rare, with 2.5-3 cases per 100,000 people per year in the Western world.
- Salivary gland malignancies account for 3-5% of all head and neck cancers.
- Different salivary glands have varying rates of malignancy.
Histological Features
- Heavy infiltration of lymphocytes and plasma cells in the glandular tissue.
- Presence of reactive lymphoid follicles without atypical lymphoid cells.
- Atrophy and loss of acini in the salivary glands.
- Encasement of glandular ducts in thick fibrous tissues.
- Periductal and interlobular sclerosis in the affected glands.
Pathogenesis
- Formation of salivary calculus or sialolith in the duct of the salivary gland.
- Abnormalities of salivary gland ducts leading to excessive accumulation or retention of secretions.
- Immune, especially autoimmune, causes with lymphoid immune cells and fibrous connective tissue in the glands.
- Presence of Immunoglobulin G4 (IgG4) associated with plasma cells infiltrating the salivary glands.
- Primarily observed in adult patients, but cases have been reported in children.
Diagnosis and Existing Treatment
- Definitive pre-operative diagnosis of Küttners tumor is challenging.
- Surgeons rely on pre-operative ultrasonography and Fine-needle aspiration cytology (FNAC) for presumptive diagnosis.
- Ultrasonogram shows a diffuse, heterogeneous zone of echo-shadows.
- FNAC reveals paucicellularity, lymphoplasmacytic infiltration, and fibrous depositions.
- Magnetic resonance imaging (MRI) has been used but results are inconclusive.
- Glandular sialadenectomy is the standard and most effective therapy.
- Steroid administration may be considered, especially in younger patients or those who refuse surgery.
- Glandular sialadenectomy is associated with low operative morbidity.
- Steroids can shrink the inflammatory lesion and reduce serum IgG4 values.
- Treatment options are evolving with better understanding of the condition.
Research Studies, Association with Other Conditions, Diagnostic Approaches, and Classification
- Research studies on chronic sclerosing sialadenitis include investigations on crocalculi, immunohistopathology, immune reactions, and similarities with other autoimmune diseases.
- Chronic sclerosing sialadenitis has been associated with idiopathic retroperitoneal fibrosis and has a close correlation with immunoglobulin G4.
- Diagnostic approaches include clinical perspective, color Doppler sonography, sonographically guided needle biopsy, and MRI.
- Chronic sclerosing sialadenitis is classified under ICD-10 code Xxx.x.
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.
Chronic sclerosing sialadenitis | |
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Specialty | Dentistry |
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.