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Cancer Med. Jan 2020

An International Multicenter Retrospective Analysis of Patients With Extranodal Marginal Zone Lymphoma and Histologically Confirmed Central Nervous System and Dural Involvement

Andrew J Sunderland, Raphael E Steiner, Musa Al Zahrani, Chelsea C Pinnix, Bouthaina Shbib Dabaja, Jillian R Gunther, Loretta J Nastoupil, Mats Jerkeman, David Joske, Gavin Cull, Tarec El-Galaly, Diego Villa, Chan Yoon Cheah

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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

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