Marginal zone B-cell lymphoma: A clinical comparison of nodal and mucosa-associated lymphoid tissue types

Purpose: In the lnternational Lymphoma Study Group classification of lympho ma, extranodal marginal zone C-cell lymphoma (MZL) of mucosa-associated lym phoid tissue (MALT) type is listed as a distinctive entity. However, nodal MZL is listed as a provisional entity because of questions as to whether it is truly a disease or just an advanced stage of MALT-type MZL, To resolve the issue of whether primary nodal MZL without involvement of mucosal sites exists and whether it is clinically different from extranodal MALT-type ly mphoma, we compared the clinical features of these two lymphomas. Patients and Methods: Five expert hematopathologists reached a consensus di agnosis of MZL in 93 patients, Seventy-three were classified as having MALT -type MZL because of involvement of a mucosal site at the time of diagnosis , and 20 were classified as having nodal MZL because of involvement of lymp h nodes without involvement of a mucosal site. Results: A comparison of the clinical features of nodal MZL and MALT-type M ZL showed that more patients with nodal MZL presented with advanced-stage d isease (71% v 34%; P = .02), peripheral lymphadenopathy (100% v 8%; P < .00 1), and para-aortic lymphadenopathy (56% v 14%; P < .001) than those with M ALT-type MZL, However, fewer patients with nodal MZL had a large mass (grea ter than or equal to 5 cm) than those with MALT-type MZL (31% v 68%; P = .0 3), The 6-year overall survival of patients with nodal MZL was lower than t hat far patients with MALT-type MZL (56% v 81%; P = .09), with a similar re sult for failure-free survival (28% v 65%; P = .01). Comparisons of patient s with International Prognostic Index scores of 0 to 3 showed that those wi th nodal MZL had lower 5-year overall survival (52% v 88%; P = .025) and fa ilure-free survival (30% v 75%; P = .007) rates than those with MALT-type M ZL, Conclusion: Nodal MZL seems to be a distinctive disease entity rather than an advanced stage of MALT-type MZL because the clinical presentations and s urvival outcomes are different in these two types of MZL. Clinically, nodal MZL is similar to other low-grade, node-based B-cell lymphomas, such as fo llicular and small lymphocytic lymphomas. (C) 1999 by American Society of C linical Oncology.