Dual institution experience of extranodal marginal zone lymphoma reveals excellent long‐term outcomes

Abstract

Extranodal marginal zone lymphoma (EMZL) is a B-cell lymphoma arising from mucosa-associated lymphoid tissue (MALT). The disease characteristics, clinical course and treatment vary considerably based on site of involvement. Because long-term outcome data for EMZL are limited, we sought to describe the clinical details of a large number of patients with EMZL evaluated at the Case Comprehensive Cancer Center over a 12-year period to identify prognostic markers including the impact of site of involvement. We identified 211 cases of EMZL involving the stomach (30%), ocular adnexa (19%), lungs (16%) and intestines (9%). Initial treatment included antibiotics (18%), radiation (21%), rituximab (20%), chemotherapy (3%), rituximab + chemotherapy (7%), surgery (17%) or observation (8%). After a median follow-up of 44·3 months (range 2·2-214·9), median progression-free survival (PFS) was 68·2 months (95% confidence interval [CI] 54·5-111·3) and median overall survival (OS) has not been reached. Age >60 years, elevated lactate dehydrogenase level (LDH), ≥4 lymph node groups involvement, and high follicular lymphoma international prognostic index (FLIPI) were associated with inferior PFS/OS. In summary, patients with EMZL have excellent prognosis with median OS in excess of 10 years. Age, elevated LDH, advanced disease, and high FLIPI score are associated with worse outcomes.

Dual institution experience of nodal marginal zone lymphoma reveals excellent long‐term outcomes in the rituximab era

“…Transformation to DLBCL occurred in 7 (12·5%) patients during the observation period, a rate of 3·9% annually, which is comparable to the rates of 6–10% observed in prior reports (Arcaini et al , ; van den Brand et al , ), to a recent series of patients with follicular lymphoma (Link et al , ) with 10·7% rate of transformation over 5 years and to a 6·6% rate of transformation in our EMZL case series (Starr et al , ). While there was no association between transformation and inferior OS, this analysis is limited by small sample size and limited observation period.…”

“…We compared the outcomes of a contemporaneous cohort of 211 patients identified as having EMZL (Starr et al , ) to this cohort of patients with NZML. After a median follow‐up of 42·3 months (range: 2·3–214·9), Kaplan–Meier estimates of median PFS was 42·4 months in the NMZL group versus 68·2 months in the EMZL group ( P = 0·032).…”

“…Additionally, there were 211 patients with EMZL, 64 patients with splenic MZL and 22 patients with typical MZL phenotype unclassifiable by anatomic site (Fig S1). We have recently reported the outcomes of patients with EMZL (Starr et al , ) and those of splenic MZL will be reported separately. Some patients had involvement of multiple lymph node groups but had predominant mucosal involvement and were categorized as EMZL, rather than NMZL, and were not included in this analysis.…”

“…Furthermore, given the paucity of data, our understanding of the behaviour of this disease with regard to treatment is often extrapolated from other indolent lymphomas, such as follicular lymphoma. Our study aims to delineate patient outcomes, prognostic factors and treatment trends among a large cohort of patients with NMZL, and compare these with our experience with EZML (Starr et al , ).…”

Splenic marginal zone lymphoma: excellent outcomes in 64 patients treated in the rituximab era

“…In addition, there were 56 patients that we identified as having nodal MZL, 211 patients as extranodal MZL, and 22 patients with typical MZL features, but who were unable to further classified. These patients have been reported separately [8].…”