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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.

  • 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)
Published Article
Cancer Medicine
Publication Date
Dec 05, 2019
DOI: 10.1002/cam4.2732
PMID: 31808316


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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