Pulmonary marginal zone lymphoma: A single center experience and review of the SEER database

INTRODUCTION: Splenic marginal zone lymphoma (SMZL) is a rare and indolent form of NHL with no standard treatment due to scarcity of clinical trials. Watch and wait approach, single agent rituximab or with chemotherapy and splenectomy are valid treatment approaches. No population-based studies examining the impact of chemotherapy and splenectomy on survival of SMZL patients are available. Thus, we decided to compare survival outcomes by clinical factors in SMZL patients deposited in the SEER database, emphasizing in the role of splenectomy on survival outcomes. METHODS: Retrospective analysis of SMZL cases in the SEER database from 1997 to 2016.SEER collects cancer incidence and survival data from population-based cancer registries covering 34% of the U.S. population. PATIENTS: SEER database was queried for SMZL patients at all ages from 1997 to 2016. Cases without histologic confirmation or no survival data were excluded. Demographic and clinical variables (age, sex, race, ethnicity, B-symptoms, Ann-Arbor stage, DLBCL transformation, first malignancy, chemotherapy with or without rituximab, splenectomy and radiotherapy) were summarized by descriptive statistics. Logistic regression used to determine variables associated with splenectomy. Overall survival (OS) and Cancer-specific survival (CSS) were the major end-points, estimated by Kaplan-Meier method. Cox regression used to calculate Hazard Ratios. RESULTS: 2212 patients met inclusion criteria, with a median age of 68yo (25-96). Patient characteristics were a follows: males (n=1152, 52.1%); whites (n=1991, 90.0%), blacks (112, 5.1%); Non-Hispanics (n=2049, 92.6%); B-symptoms (n=461, 20.8%); first malignancy (n=1754, 79.3%); Ann-Arbor Stage I (429, 19.4%), Stage IV (n=1351, 61.1%); DLBCL transformation (n=84, 3.8%). Adjusted Odds ratio (aOR) were used to compare splenectomy versus non-splenectomy treated patients: ≥60yo (aOR 0.49;0.40-0.62), males (aOR 0.77;0.64-0.93). Median survival of all the patients was 120 months (95%CI:111-132); On univariate analysis: shorter OS was significantly associated with age ≥60(HR 3.55;2.87-4.38) and non-first malignancy(HR 1.6;1.36-1.88), while shorter CSS was significantly associated with age ≥60(HR 1.76;1.33-2.34), B-symptoms (HR 1.93;1.44-2.60), Stage IV (HR 1.62;1.15-2.28), DLBCL transformation (HR 2.27;1.55-3.33) and treatment with chemotherapy (HR 1.54;1.22-1.94). On multivariate analysis, shorter OS was significantly associated with age ≥60(HR 3.52;2.83-4.37), male sex (HR 1.15;1.00-1.33), B-symptoms (HR 1.23;1.01-1.51) and non-first malignancy (HR 1.42;1.20-1.67). Shorter CSS was significantly associated with age ≥60(HR 2.15;1.61-2.87), Hispanic ethnicity (HR 1.44;1.00-2.07), B-symptoms (HR 1.77;1.30-2.39), DLBCL transformation (HR 2.03;1.38-2.99) and treatment with chemotherapy (HR 1.38;1.07-1.77). Ann Arbor staging and splenectomy were not associated with OS or CSS. CONCLUSIONS: Older age, Hispanic ethnicity, B-symptoms, DLBCL transformation, and treatment with chemotherapy were associated with poor lymphoma-specific survival. Although, age<60 and female gender were associated higher odds to undergo splenectomy, splenectomy had no impact on the risk of lymphoma-related death or OS. This retrospective study demonstrates that chemotherapy treatment (excluding rituximab) was associated with inferior outcomes in SMZL, while advanced Ann-Arbor stage was not associated with shorter survival as is observed in other indolent lymphomas that commonly present at advanced stage. Table Disclosures Alderuccio: Targeted Oncology: Honoraria; Foundation Medicine: Other: Immediate family member; Agios: Other: Immediate family member; Puma Biotechnology: Other: Immediate family member; OncLive: Consultancy; Inovio Pharmaceuticals: Other: Immediate family member. Lossos:NIH: Research Funding; Seattle Genetics: Membership on an entity's Board of Directors or advisory committees; Janssen Scientific: Membership on an entity's Board of Directors or advisory committees.

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Survival with splenectomy in splenic marginal zone lymphoma: Analysis of the Surveillance, Epidemiology and End Results (SEER) database.

Journal of Clinical Oncology ◽

10.1200/jco.2012.30.15_suppl.8044 ◽

2012 ◽

Vol 30 (15_suppl) ◽

pp. 8044-8044

Author(s):

Adam J. Olszewski

Keyword(s):

Propensity Score ◽

Survival Data ◽

Marginal Zone ◽

Relative Survival ◽

Marginal Zone Lymphoma ◽

Splenic Marginal Zone Lymphoma ◽

Primary Therapy ◽

Seer Database ◽

The Impact

8044 Background: The role of splenectomy as the primary therapy for splenic marginal zone lymphoma has been questioned. We studied relative survival in SMZL and the impact of splenectomy on lymphoma-specific survival (LSS). Methods: SMZL cases (diagnosed in 1993-2008) were derived from the SEER database. Age, sex and race-matched actuarial survival data were summarized. Factors predictive of splenectomy were studied using logistic regression with a subsequent propensity score (PS)-weighted survival analysis. Results: 1071 patients were identified with a median age of 69 years (range, 25-96) and a median follow up of 33 months. 53% were women and 92% were white. 70% of the lymphomas were stage III/IV with B symptoms recorded in 22.3%. 54% of patients underwent splenectomy. The significant factors predictive of surgery included younger age (p<10-14), stage I/II (p<10-15), B-symptoms (p=0.003), no prior malignancy (p=0.002), with significantly lower rates in black patients (OR 0.41, 95%CI 0.21-0.80, p=0.008), on the Pacific Coast (OR 0.62, 95%CI 0.47-0.81, p=0.0004) and with decreasing rates over time (p=0.0002). The actuarial 5-year relative survival was 82.8% (95%CI 77.9-86.7%) with no difference by sex (p=0.79), race or stage. 54% of deaths were related to SMZL with the estimated LSS of 80.8% (95%CI 78-84%). Splenectomy was not associated with improved LSS in propensity score-stratified log-rank test (p=0.60) or in the PS-weighted Cox model (HR=0.93, 95%CI 0.62-1.38, p=0.70). Advancing age (HR 1.05, 95%CI 1.03-1.07, p<10-8) and presence of B-symptoms (HR=1.98, 95%CI 1.30-3.01, p=0.002) were significantly predictive of death from SMZL, with no evidence of improvement in LSS over the years (HR=0.97, 95%CI=0.91-1.03, p=0.34). There was also no detectable impact of splenectomy on survival in patients who ultimately died of SMZL (p=0.83). Conclusions: In this cohort, the largest studied to date, splenectomy did not demonstrate a survival benefit in SMZL. Patients continue to have decreased survival despite advances in other indolent B-cell lymphomas. The role of splenectomy versus chemoimmunotherapy remains to be determined.

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