Treatments and Outcomes in Stage I Extranodal Marginal Zone Lymphoma in the United States

SIMPLE SUMMARY: Extranodal marginal zone lymphoma (EMZL) is a rare disease commonly diagnosed at an early stage and remains localized for prolonged periods of time. This unique characteristic makes the use of local therapies, such as radiation therapy (RT), the preferred approach. Excellent results were previously reported implementing RT; however, majority of these studies included a small number of patients, and treatment patterns in the United States are presently unknown. Furthermore, EMZL may arise in various organs, and whether the survival is similar at different locations is unclear. In the present study, we assessed the Surveillance, Epidemiology, and End Results (SEER) database aiming to examine management and survival of localized EMZL. While differences in survival were observed by primary disease location, similar survival was observed in RT-treated stage I EMZL patients and general U.S. population matched by sex, age, and calendar year. ABSTRACT: A considerable number of patients with extranodal marginal zone lymphoma (EMZL) are diagnosed with stage I disease. Information on treatments and survival by primary location remains limited. We extracted data from the Surveillance, Epidemiology, and End Results (SEER) database to assess treatment, primary location, and survival of patients with stage I EMZL. Results show that 7961 patients met inclusion criteria. Observation (no treatment) was the most common approach (31%) followed by radiation therapy (RT, 23%). The median overall survival (OS) was 17.3 years (95%CI 16.3 to 18.3). Shorter survival was observed in patients with stage I EMZL compared to expected survival in a cohort derived from the general U.S. population matched by sex, age, and calendar year at diagnosis. However, similar survival was observed in RT-treated patients. We identified age ≥ 60 years (SHR = 4.00, 95%CI 3.10–5.15; p < 0.001), higher grade transformation (SHR = 4.63, 95%CI 3.29–6.52; p < 0.001), and primary lung EMZL (SHR = 1.44, 95%CI 1.05–1.96; p = 0.022) as factors associated with shorter lymphoma-specific survival (LSS). Conversely, primary skin location (SHR = 0.50, 95%CI 0.33–0.77; p = 0.002) was associated with longer LSS. Our results support the use of RT as the preferred approach in localized EMZL.