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

Authors
  • Sunderland, Andrew J1
  • Steiner, Raphael E2
  • Al Zahrani, Musa3, 4
  • Pinnix, Chelsea C5
  • Dabaja, Bouthaina Shbib5
  • Gunther, Jillian R5
  • Nastoupil, Loretta J2
  • Jerkeman, Mats6
  • Joske, David1, 7, 8
  • Cull, Gavin1, 7, 8
  • El-Galaly, Tarec9
  • Villa, Diego3
  • Cheah, Chan Yoon1, 7, 8
  • 1 Department of Haematology, Sir Charles Gairdner Hospital, Nedlands, WA, Australia. , (Australia)
  • 2 Department of Lymphoma/Myeloma, MD Anderson Cancer Center, Houston, TX, USA.
  • 3 University of British Columbia and BC Cancer Centre for Lymphoid Cancer, Vancouver, British Columbia, Canada. , (Canada)
  • 4 Department of Medicine, King Saud University Hospital, Riyadh, Saudi Arabia. , (Saudi Arabia)
  • 5 Department of Radiation Oncology, MD Anderson Cancer Center, Houston, TX, USA.
  • 6 Skånes University Hospital, Lund University, Lund, Sweden. , (Sweden)
  • 7 Department of Haematology, Pathwest Laboratory Medicine WA, Nedlands, WA, Australia. , (Australia)
  • 8 Medical School, University of Western Australia, Crawley, WA, Australia. , (Australia)
  • 9 Department of Hematology, Aalborg University Hospital, Aalborg, Denmark. , (Denmark)
Type
Published Article
Journal
Cancer Medicine
Publisher
Wiley
Publication Date
Dec 05, 2019
Identifiers
DOI: 10.1002/cam4.2732
PMID: 31808316
Source
Medline
Keywords
Language
English
License
Unknown

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. © 2019 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.

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