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Prepublished online as a Blood First Edition Paper on November 27, 2002; DOI 10.1182/blood-2002-04-1279.
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Blood, 1 April 2003, Vol. 101, No. 7, pp. 2489-2495
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Nongastric marginal zone B-cell lymphoma of mucosa-associated
lymphoid tissue
Emanuele Zucca,
Annarita Conconi,
Ennio Pedrinis,
Sergio Cortelazzo,
Teresio Motta,
Mary K. Gospodarowicz,
Bruce J. Patterson,
Andrés J. M. Ferreri,
Maurilio Ponzoni,
Liliana Devizzi,
Roberto Giardini,
Graziella Pinotti,
Carlo Capella,
Pier Luigi Zinzani,
Stefano Pileri,
Armando López-Guillermo,
Elias Campo,
Achille Ambrosetti,
Luca Baldini, and
Franco Cavalli
From the International Extranodal Lymphoma Study Group
(IELSG). A complete list of the investigators of the IELSG appears in
"Appendix 1" and "Appendix 2."
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.
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