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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.

Pediatric Parotid Chronic Sclerosing Sialadenitis in an African-American Female: A Rare Case and Review of the Literature