Küttner’s Tumour - Chronic Sclerosing Sialadenitis of the Submandibular Gland

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Küttner’s Tumour - Chronic Sclerosing Sialadenitis of the Submandibular Gland

Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2017, Vol 1, Issue 6

Abstract

Chronic sclerosing sialadenitis, originally described by H. Küttner in 1896, is a rare cause of salivary gland enlargement [1]. Its clinical and radiographic presentations can mimic other conditions such as, lymphoma or infectious causes and can only be correctly diagnosed following removal of the gland. This “Küttner’s Tumour” is now classified as an IgG4 related disease, with characteristic dense infiltrate of immunoglobulin (Ig) G4- positive plasma cells. However, the exact aetiology of IgG4 related disease remains unknown with no known role of the IgG4 molecule itself [2]. Other IgG 4 related diseases include Riedel‘s thyroiditis, Mikulicz’s disease and idiopathic retroperitoneal fibrosis [3]. This case demonstrates the diagnostic difficulty of this presentation, with multiple differential diagnoses that cannot be excluded until the final histopathological examination. The patient was a 63-year-old man who presented with bilateral submandibular swelling, which were present for four months. Physical examination showed firm, non-tender enlarged submandibular glands. The laboratory values showed a normal leukocyte count (10.6 x 109/L), haemoglobin level 14.2 g/dL), and platelet count (292 x 109/L). Nasoendoscopy examination to the level of the vocal cords was unremarkable. Ultrasound showed the submandibular glands contained a number of hypoechoic nodules which were well defined with attenuated echogenic strands internally. Fine needle aspiration cytology showed a mixed population of lymphoid cells. Contrast-enhanced computerised tomography scan revealed bilateral asymmetrical enlargement of right and left submandibular glands (Figure 1). No sialolithiasis was found. There was no significant cervical lymphadenopathy. As the diagnosis of lymphoma could not be excluded a decision was made to remove the right submandibular gland. Final pathology revealed the submandibular gland measured 55x38x25 mm. Histologically the lesion was composed of well-defined areas showing stromal fibrosis associated with acinar atrophy, florid chronic inflammation that includes conspicuous germinal centres, but also prominent numbers of interstitial plasma cells and vascular changes (Figures 2 & 3).Chronic sclerosing sialadenitis (Küttner’s tumour) is a nonneoplastic, chronic inflammatory disease of the submandibular gland first described by Küttner in 1896 [1]. A predominance of male sex has been recognised in the sixth to eighth decades [4]. Two factors appear important in the aetiology of Küttner’s tumour: an initial disturbance in saliva flow causing an obstructive sialadenitis and an immune reaction of the salivary duct system causing an obstructive progressive sialadenitis. Chronic sclerosing sialadenitis has been classified into four histological stages depending on the degree of inflammation; a. Stage 1: focal sialadenitis; b. Stage 2: diffuse lymphocytic sialadenitis with salivary gland fibrosis; c. Stage 3: chronic sclerosing sialadenitis with salivary gland sclerosis; d. Stage 4: chronic progressive sialadenitis with salivary gland cirrhosis [4]; Histologically, the disease is characterised by acinar atrophy, dense lymphocytic infiltration and fibrosis [5,6].

Authors and Affiliations

CM Bowe, C Wan, T Walker, S Walsh

Keywords

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EP ID EP581144
DOI 10.26717/BJSTR.2017.01.000525
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