title: 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.
authors.affiliation: University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Adam G
authors.initials: AG
authors.lastname: Starr
authors.affiliation: University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Paolo F
authors.initials: PF
authors.lastname: Caimi
authors.affiliation: University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: PingFu
authors.initials: P
authors.lastname: Fu
authors.affiliation: University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Mira R
authors.initials: MR
authors.lastname: Massoud
authors.affiliation: University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Howard
authors.initials: H
authors.lastname: Meyerson
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Eric D
authors.initials: ED
authors.lastname: Hsi
authors.affiliation: University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: David B
authors.initials: DB
authors.lastname: Mansur
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Sheen
authors.initials: S
authors.lastname: Cherian
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Arun D
authors.initials: AD
authors.lastname: Singh
authors.affiliation: University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Brenda W
authors.initials: BW
authors.lastname: Cooper
authors.affiliation: University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Marcos J G
authors.initials: MJ
authors.lastname: De Lima
authors.affiliation: University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Hillard M
authors.initials: HM
authors.lastname: Lazarus
authors.affiliation: University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Stanton L
authors.initials: SL
authors.lastname: Gerson
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Deepa
authors.initials: D
authors.lastname: Jagadeesh
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Mitchell R
authors.initials: MR
authors.lastname: Smith
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Robert M
authors.initials: RM
authors.lastname: Dean
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Brad L
authors.initials: BL
authors.lastname: Pohlman
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Brian T
authors.initials: BT
authors.lastname: Hill
authors.affiliation: University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Basem M
authors.initials: BM
authors.lastname: William
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000032:analysis; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007770:L-Lactate Dehydrogenase
keywords: B cells; MALT; extranodal lymphoma; mucosal; non-Hodgkin lymphoma