Prognosis of Marginal Zone Lymphoma (MZL)

5-year Relative Survival

The 5-year relative survival for MZL is reported by the National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) database (n=9805).1

5-year relative survival (%)1


Prognostic Indices

NHL subtype, disease stage, and disease grade are all associated with each patient’s prognosis.2 Indices have been created that combine the relative input of disease stage and other prognostic factors—including age, ECOG performance status, and serum LDH—into a single score that helps determine prognosis.2 Prognostic indices vary between lymphoma subtypes; separate indices have been developed for aggressive NHL (like DLBCL), follicular lymphoma (FL), and mantle cell lymphoma (MCL), but not marginal zone lymphoma (MZL).2 However, the Follicular Lymphoma International Prognostic Index, or FLIPI score has been demonstrated to have strong prognostic value in patients with extranodal or splenic MZL.3

Histologic Transformation

MZL may transform into aggressive NHL, most often DLBCL. The risk of histologic transformation is low, but is associated with a worse clinical outcome.4-6 Pathologic evaluation for histologic transformation should include FISH for BCL6 and MYC rearrangements.2

DLBCL, diffuse large B-cell lymphoma; ECOG, Eastern Cooperative Oncology Group; FISH, fluorescence in situ hybridization; FLIPI, Follicular Lymphoma International Prognosis Index.

References: 1. National Cancer Institute. SEER Cancer Statistics Review 2008-2014: Table 19.29. https://seer.cancer.gov/archive/csr/1975_2015/results_merged/sect_19_nhl.pdf. Accessed July 1, 2019. 2. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for B-Cell Lymphomas V.4.2019. © National Comprehensive Cancer Network, Inc. 2019. All rights reserved. Accessed July 10, 2019. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way. 3. Heilgeist A et al. Prognostic value of the Follicular Lymphoma International Prognostic Index score in marginal zone lymphoma: an analysis of clinical presentation and outcome in 144 patients. Cancer. 2013;119(1):99-106. 4. Conconi A, Franceschetti S, van Hohenstaufen A, et al. Histologic transformation in marginal zone lymphomas. Ann Oncol. 2015; 26(11):2329-2335. 5. Alderuccio JP, Zhao W, Desai A, et al. Risk Factors for Transformation to Higher-Grade Lymphoma and Its Impact on Survival in a Large Cohort of Patients With Marginal Zone Lymphoma From a Single Institution. J Clin Oncol. 2018;36(15_suppl):7559-7559.