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Multicenter Study
. 2020 Jan;9(2):663-670.
doi: 10.1002/cam4.2732. Epub 2019 Dec 5.

An international multicenter retrospective analysis of patients with extranodal marginal zone lymphoma and histologically confirmed central nervous system and dural involvement

Andrew J Sunderland  1 , Raphael E Steiner  2 , Musa Al Zahrani  3   4 , Chelsea C Pinnix  5 , Bouthaina Shbib Dabaja  5 , Jillian R Gunther  5 , Loretta J Nastoupil  2 , Mats Jerkeman  6 , David Joske  1   7   8 , Gavin Cull  1   7   8 , Tarec El-Galaly  9 , Diego Villa  3 , Chan Yoon Cheah  1   7   8
Affiliations
Multicenter Study

An international multicenter retrospective analysis of patients with extranodal marginal zone lymphoma and histologically confirmed central nervous system and dural involvement

Andrew J Sunderland et al. Cancer Med. 2020 Jan.

Abstract

Marginal zone lymphoma of the central nervous system (CNS MZL) is rare. The clinical features, treatment, and prognosis are not well characterized. We performed a multicenter retrospective study of CNS MZL. Twenty-six patients were identified: half with primary and half with secondary CNS involvement. The median age was 59 years (range 26-78), 62% female and 79% with ECOG performance status ≤ 1. The most common disease site was the dura (50%). Treatment was determined by the treating physician and varied substantially. After a median follow up of 1.9 years, the estimated 2-year progression-free (PFS) and overall survival (OS) rates were 59% and 80%, respectively. Secondary CNS MZL was associated with 2-year OS of 58%. CNS MZL is rare, but relative to other forms of CNS lymphoma, outcomes appear favorable, particularly among the subset of patients with dural presentation and primary CNS presentation.

Keywords: CNS lymphoma; marginal zone lymphoma.

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Conflict of interest statement

DV: consulting/advisory/honoraria—Roche, Janssen, Celgene, Lundbeck, Gilead, AstraZeneca, Abbvie, Seattle Genetics, Nanostring. CYC: consulting/advisory/honoraria—Roche, Janssen, Takeda, MSD, Gilead, Bristol Myers Squibb, AstraZeneca; research funding—Celgene, Roche, Abbvie; travel expenses—Roche, Amgen. MJ: consulting/advisory/honoraria—Roche, Janssen, Acerta, Gilead; research funding—Celgene, Roche, Abbvie, Janssen, Gilead. TEG: Travel funding Roche and Takeda, Employed by Roche, Basel, 1st January 2019. LJN: honoraria: Bayer, Celgene, Genentech, Gilead, Janssen, Juno, Novartis, Spectrum, TG Therapeutics. Research funding: Celgene, Genentech, Janssen, Karus Therapeutics, Merck, TG Therapeutics.

Figures

Figure 1
Figure 1
A, Kaplan‐Meir estimate of PFS (in years), B, Kaplan‐Meir estimate of OS (in years)
See this image and copyright information in PMC

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