We describe five cases of mucosa-associated lymphoid tissue (MALT) lymphoma in patients with Sjögren's syndrome with emphasis on the initial and follow-up imaging features.
In three patients, the lymphoma initially arose in the salivary gland, and in two patients, the ocular adnexa. Two patients received no treatment but did have close follow-up for 15 months and 2 years, respectively; no progression of the disease was observed. In the other three patients, although complete remission was achieved in the primary site, the disease spread to other mucosal sites during follow-up periods of 1, 3, and 9 years, respectively. Our findings show that MALT lymphoma in patients with Sjögren's syndrome tends to originate in the organs targeted by Sjögren's syndrome, and, despite the indolent nature of MALT, the lymphoma tends to spread to other mucosal sites during the follow-up period.