Nongastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue.
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.
Zucca
Emanuele
E
Oncology Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Switzerland. ielsg@ticino.com
Conconi
Annarita
A
Pedrinis
Ennio
E
Cortelazzo
Sergio
S
Motta
Teresio
T
Gospodarowicz
Mary K
MK
Patterson
Bruce J
BJ
Ferreri
Andrés J M
AJ
Ponzoni
Maurilio
M
Devizzi
Liliana
L
Giardini
Roberto
R
Pinotti
Graziella
G
Capella
Carlo
C
Zinzani
Pier Luigi
PL
Pileri
Stefano
S
López-Guillermo
Armando
A
Campo
Elias
E
Ambrosetti
Achille
A
Baldini
Luca
L
Cavalli
Franco
F
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