A case of Sjogren syndrome coexistent with MALT lymphoma occurring along the parotid gland and trachea

Abstract A 62-year-old woman with Sjogren syndrome was admitted for computed tomographic (CT) evaluation of a thickened trachea and parotid tumor. She had been given a diagnosis of mucosa-associated lymphoid tissue (MALT) lymphoma 6 years previously, and had undergone surgical resection of the parotid tumor. Endoscopic examination revealed an annular tumor that had formed a stricture in the mid-trachea. Pathologic specimens were obtained by surgical resection of the parotid tumor and bronchoscopic biopsy of the tracheal tumor. Both histological examinations revealed MALT-type marginal zone B-cell lymphoma. Because CD20 immunostaining was positive, the patient received 6 cycles of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) without any signs of major toxicity. All lesions disappeared after treatment, and this patient remained disease-free for 40 months.