Clinical characteristics and survival outcomes of thymic mucosa-associated lymphoid tissue lymphoma: A multicenter analysis of 82 patients.

[BACKGROUND] The systemic study of thymic mucosa-associated lymphoid tissue (MALT) lymphoma remains lacking. The objective of this study was to characterize the clinical features and outcomes of patients with thymic MALT lymphoma.

[METHODS] The authors conducted a retrospective analysis of patients with thymic MALT lymphoma who were diagnosed at multiple medical centers in China from 2011 to 2024. Clinical, laboratory, therapeutic, and survival data were collected.

[RESULTS] In total, 82 patients were included. The median age of the cohort was 47 years with a female predominance (female/male ratio = 5.3:1.0). Eighty-two percent of patients presented with limited stage disease. Forty-one patients had a definitive diagnosis of autoimmune disease, with Sjogren's syndrome being the most common (n = 37). Antinuclear antibodies were positive in 42 of 44 (95%) of examined patients. Management strategies included watch and wait (2%), local therapy alone (surgery, radiotherapy, or both; 71%), a combination of local and systemic therapy (26%), or systemic therapy alone (1%). With a median follow-up of 2.7 years, the 2-year progression-free and overall survival rates for the entire cohort were 98.6% (95% confidence interval, 95.8%-100.0%) and 100% (95% confidence interval, 100.0%-100.0%), respectively. Patients treated with local therapy alone (n = 58) and those who received local and systemic therapy (n = 21) had similar progression-free survival (p = .530). Patients who underwent surgery alone (n = 53) had a 2-year progression-free survival rate of 100% (95% confidence interval, 100.0%-100.0%).

[CONCLUSIONS] Patients with thymic MALT lymphoma have an excellent prognosis. Sjogren's syndrome is closely associated with thymic MALT lymphoma. Routine screening for autoimmune disorders and surgical resection are recommended for patients who have newly diagnosed thymic MALT lymphomas.