title: Primary Sjögren's syndrome initially presenting as submandibular mucosa-associated lymphoid tissue lymphoma: A case report.
abstract: The present study reports the case of a 24-year-old female affected with primary Sjögren's syndrome (pSS), who presented with mucosa-associated lymphoid tissue (MALT) lymphoma of the submandibular gland. Reports of such cases, particularly in young patients, are very rare. The patient, who presented no oral or ocular symptoms prior to the development of the mass, underwent surgical ablation of the gland, and MALT lymphoma was diagnosed by histopathology. Since MALT lymphoma in the submandibular gland is rarely observed in otherwise healthy young females, a rheumatologist and an oncologist were consulted. Following a number of immunological tests, the results of the Schirmer's and Saxon tests were negative. However, the antinuclear antibody test revealed a speckled appearance, and there was also strong positivity for the serological markers of Sjögren's syndrome. Consequently, pSS was diagnosed, despite the fact that the patient did not fulfill all the diagnostic criteria for the disease. Therefore, MALT lymphoma in a single salivary gland should be used as a differential diagnosis for Sjögren's syndrome in young asymptomatic patients. Additionally, a multidisciplinary team is required for the treatment and management of these patients.
authors.affiliation: Department of Otolaryngology - Head and Neck Surgery, China Medical University and Hospital, Taichung 40402, Taiwan, R.O.C.
authors.forename: Liang-Yu
authors.initials: LY
authors.lastname: Chen
authors.affiliation: Department of Otolaryngology - Head and Neck Surgery, China Medical University and Hospital, Taichung 40402, Taiwan, R.O.C.; Graduate Institute of Clinical Medical Science, China Medical University, Taichung 40402, R.O.C.
authors.forename: Ming-Hsui
authors.initials: MH
authors.lastname: Tsai
authors.affiliation: Department of Otolaryngology - Head and Neck Surgery, China Medical University and Hospital, Taichung 40402, Taiwan, R.O.C.
authors.forename: Li-Tai
authors.initials: LT
authors.lastname: Tsai
authors.affiliation: Department of Psychology, Asia University, Taichung 41354, Taiwan, R.O.C.
authors.forename: Hsin-Man
authors.initials: HM
authors.lastname: Lu
authors.affiliation: Department of Pathology, China Medical University Hospital, Taichung 40402, Taiwan, R.O.C.; Department of Pathology, China Medical University, Beigang Hospital, Beigang, Yunlin 65152, R.O.C.; Department of Dentistry, National Yang-Ming University, Taipei 11221, Taiwan, R.O.C.
authors.forename: Chia-Ing
authors.initials: CI
authors.lastname: Jan
keywords: Sjögren's syndrome; mucosa associated lymphoid tissue lymphoma; submandibular swelling