Abstract
Chronic sclerosing sialadenitis (CSS) or Küttner tumor is an under-recognized, benign fibroinflammatory disease most commonly seen in the submandibular gland of older adult males. Sialolithiasis or bacterial infection was first suspected as an etiology, but CSS is now considered an immunoglobulin G4-related disease (IgG4-RD). IgG4-RD can affect almost every organ in the body, characterized by organ fibrosis with IgG4-positive plasmacytes. Numerous autoimmune-related diseases have been unified under IgG4-RD, including Mikulicz disease (MD), autoimmune pancreatitis, Reidel’s thyroiditis, and others. In any organ, IgG4-RD can present similar to malignancy. Due to the ability to mimic malignancy, it is crucial to be aware of this under-recognized clinical entity. CSS is currently of broad and high clinical interest due to increased understanding, multiorgan involvement, and more clearly defined criteria. To increase awareness of this disease, we describe a rare presentation of CSS with a literature review.
Highlights
Chronic sclerosing sialadenitis (CSS) or Küttner tumor is an uncommonly recognized, benign fibroinflammatory disease of the major salivary glands
Numerous autoimmune-related diseases have been unified under the umbrella of Immunoglobulin G4-related disease (IgG4-RD), including Mikulicz disease (MD), autoimmune pancreatitis, retroperitoneal fibrosis (Ormond’s disease), Reidel’s thyroiditis, and others [5]
In an attempt to further increase awareness of this underrecognized disease, we present, to the best of the author's knowledge, the youngest female presenting with isolated CSS of the parotid gland
Summary
Chronic sclerosing sialadenitis (CSS) or Küttner tumor is an uncommonly recognized, benign fibroinflammatory disease of the major salivary glands. It primarily occurs in the submandibular gland of middle-aged and elderly adults, typically affecting males more than females [1]. The majority of pediatric salivary gland tumors are benign, when a parotid mass is discovered in a pediatric patient, it can be a more ominous sign. The tumor showed a retained lobular architecture with marked fibrosis, acinar atrophy, and duct dilation along with moderate chronic inflammatory infiltrates of lymphocytes and some plasma cells. A - Section of the tumor with moderate chronic inflammatory infiltrates composed predominantly of lymphocytes with some plasma cells; B - Normal parotid salivary gland; C Salivary gland tissue retaining the lobular architecture with marked fibrosis, acinar atrophy, and duct dilation; D - Section of the tumor with chronic inflammatory infiltrates; E - Section of the tumor with some ducts showing squamous metaplasia; F - Section showing a dilated duct filled with mucinous debris
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