Head and neck amyloidosis – a report on five cases
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Background: Amyloidosis is a group of diseases caused by the extracellular accumulation of insoluble fibers called amyloid in the tissues and organs. They have a secondary beta-sheet structure, which makes them resistant to proteolysis. In histological examination amyloid deposits stain with Congo red and show an apple-green birefringence in polarized light. Amyloid deposits disturb the function of organs and cause clinical symptoms. Their formation or accumulation in the system may be acquired or inherited. Due to the location of amyloid deposits we distinguish systemic and localized amyloidosis with the formation of tumors (usually from light chains). Case reports: 5 cases of amyloidosis in the head and neck region are presented in this paper. The locations of the amyloid deposits were as follows: larynx, nasopharynx, sublingual and submandibular gland and the tongue. The initial clinical presentation correlated with location of amyloid tumour in our patients. Two patients had history of local recurrence of the disease. Surgical resection and histopathological examination were performed. Sections stained with Congo red confirmed the diagnosis of amyloidosis. Three patients had potential conditions predisposing to amyloidosis: previous radiotherapy, chronic inflammation due to hepatitis C virus infection and graft versus host disease. Conclusion: Amyloidosis should be considered as the cause of symptoms in pathologies of the head and neck region. The diagnosis requires a histopathological examination. The systemic form of the disease must be ruled out in all patients with head and neck amyloidosis. In localized amyloidosis the surgical resection of the lesions is the procedure of choice, however the organ’s functionality should be taken into account.
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