title: Nongastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue.
abstract: A retrospective survey of patients with pathologically reviewed extragastric mucosa-associated lymphoma tissue (MALT) lymphomas from 20 institutions was performed. A total of 180 patients with histologically confirmed diagnosis of extragastric MALT lymphomas were studied. Their median age was 59 years (range, 21-92 years). Ann Arbor stage I disease was present in 115 patients (64%) and stage II disease in 16 (9%). Most cases were in the low or low-intermediate risk groups according to the International Prognostic Index (IPI). Forty-one (23%) patients had involvement of more than one extranodal site at diagnosis and in 24 cases (13%) the lymphoma presented at multiple mucosal sites (9 of them with only mucosal involvement, without bone marrow or nodal disease). Lymph node involvement was present in 21%. Patients were treated with a variety of therapeutic strategies, including chemotherapy in 78 cases. The median overall survival (OS) was not reached; the 5-year OS rate was 90% (95% CI, 82%-94%), the 5-year cause-specific survival (CSS) was 94% (95% CI, 87%-97%), and the 5-year progression-free survival (PFS) was 60% (95% CI, 50%-70%). Multivariate analysis showed that Ann Arbor stage was significantly associated with longer OS, nodal involvement with longer CSS, and favorable IPI score with better PFS. At a median follow-up of 3.4 years, 48 patients (27%; 95% CI, 20%-34%) had a relapse, 6 (3%; 95% CI, 1%-7%) showed histologic transformation, and 18 (10%; 95% CI, 6%-15%) experienced the development of a second tumor. Our data confirm the indolent nature of nongastric MALT lymphomas and the high rate of patients presenting with disseminated disease, which, when limited to mucosal sites, was not associated with a poorer outcome.
authors.affiliation: Oncology Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Switzerland. ielsg@ticino.com
authors.forename: Emanuele
authors.initials: E
authors.lastname: Zucca
authors.forename: Annarita
authors.initials: A
authors.lastname: Conconi
authors.forename: Ennio
authors.initials: E
authors.lastname: Pedrinis
authors.forename: Sergio
authors.initials: S
authors.lastname: Cortelazzo
authors.forename: Teresio
authors.initials: T
authors.lastname: Motta
authors.forename: Mary K
authors.initials: MK
authors.lastname: Gospodarowicz
authors.forename: Bruce J
authors.initials: BJ
authors.lastname: Patterson
authors.forename: Andrés J M
authors.initials: AJ
authors.lastname: Ferreri
authors.forename: Maurilio
authors.initials: M
authors.lastname: Ponzoni
authors.forename: Liliana
authors.initials: L
authors.lastname: Devizzi
authors.forename: Roberto
authors.initials: R
authors.lastname: Giardini
authors.forename: Graziella
authors.initials: G
authors.lastname: Pinotti
authors.forename: Carlo
authors.initials: C
authors.lastname: Capella
authors.forename: Pier Luigi
authors.initials: PL
authors.lastname: Zinzani
authors.forename: Stefano
authors.initials: S
authors.lastname: Pileri
authors.forename: Armando
authors.initials: A
authors.lastname: López-Guillermo
authors.forename: Elias
authors.initials: E
authors.lastname: Campo
authors.forename: Achille
authors.initials: A
authors.lastname: Ambrosetti
authors.forename: Luca
authors.initials: L
authors.lastname: Baldini
authors.forename: Franco
authors.initials: F
authors.lastname: Cavalli
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002423:Cause of Death; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D018442:Lymphoma, B-Cell, Marginal Zone / Q000401:mortality* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging / Q000401:mortality; D016609:Neoplasms, Second Primary / Q000401:mortality; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D015996:Survival Rate