Abstract

Sjögren’s syndrome (SS) is a systemic chronic autoimmune disorder characterized by lymphocytic infiltration of the exocrine glands, as well as oral and ocular dryness. Among the late complications, malignant lymphoma is the most serious complication of SS. The risk of lymphoma in patients with SS has been estimated to be approximately 7–19 times higher than that in a generally healthy population. Although various histologic subtypes of lymphoma can occur in patients with SS, mucosa-associated lymphoid tissue (MALT) lymphoma accounts for 48–75% of malignant lymphomas that are frequently located in the parotid gland. However, MALT lymphoma affecting the tongue in patients with SS is extremely rare. Here, we share our experience with a unique case of MALT lymphoma of the tongue, originating from the minor salivary gland tissue in a patient with SS. Through this case report, we emphasize that MALT lymphoma should be considered in the differential diagnosis of a tongue mass in patients with SS.

Highlights

  • Sjögren’s syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, such as the salivary and lacrimal glands

  • mucosaassociated lymphoid tissue (MALT) lymphomas are rarely observed in the minor salivary glands [3–6]

  • During the preoperative diagnostic work-up, serologic tests to evaluate Sjögren’s syndrome were performed as follows: the anti-nuclear antibody (ANA) and anti-SS-A (Ro) antibody test results were positive with a titer of 1:1280 for ANA antibody and with a level of >200 U/mL for anti-SS-A (Ro) antibody, whereas the anti-SS-B (La) antibody and rheumatoid factor (RF) test results were negative

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Summary

Introduction

Sjögren’s syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, such as the salivary and lacrimal glands. As the exocrine glands are destroyed, the production of saliva and lacrimal fluid is reduced, causing dry eyes and mouth. This condition is clinically diagnosed when specific serum autoantibodies are detected. 5% of patients with SS develop B-cell non-Hodgkin lymphoma (NHL), and the majority are mucosaassociated lymphoid tissue (MALT) lymphomas, which are commonly encountered in the parotid gland. MALT lymphomas are rarely observed in the minor salivary glands [3–6]. To the best of our knowledge, this is the first report of a MALT lymphoma occurring in the minor salivary gland of the tongue in a patient with SS. We performed a review of the relevant literature regarding this disease for the accurate diagnosis and appropriate treatment of patients with SS with a tongue mass

Case Report
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