Originally published as JCO Early Release 10.1200/JCO.2005.17.624 on July 11 2005

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Clinicopathologic and Genetic Profile of Intracranial Marginal Zone Lymphoma: A Primary Low-Grade CNS Lymphoma That Mimics Meningioma

From the Division of Neuropathology and Section of Hematopathology, Washington University School of Medicine, St Louis, MO; Division of Neuropathology and Department of Neurology, Mayo Clinic, Rochester, MN; Department of Pathology, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA; Division of Neurosurgery, University of Toronto, Toronto, Ontario, Canada; Division of Neuropathology, University of Florida College of Medicine, Gainesville, FL; and Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD

Address reprint requests to Pang-hsien Tu, MD, PhD, Division of Neuropathology, Campus Box 8118, Washington University School of Medicine, St Louis, MO 63110-1093; e-mail: btu{at}path.wustl.edu

PURPOSE: Although rare overall, marginal zone B-cell lymphoma (MZBCL) is the most common primary low-grade CNS lymphoma reported in the literature. The aim of this study is to elucidate the biology and genetic features of this unusual tumor.

PATIENTS AND METHODS: Fifteen CNS MZBCLs were studied clinically, pathologically, and genetically, including fluorescent in situ hybridization analyses with commercially available MALT1 and IgH break-apart and centromere 3, 7, 12, and 18 probes.

RESULTS: CNS MZBCLs preferentially affect middle-aged women (female-to-male ratio, 4:1), with 93% presenting as dural-based masses mimicking meningioma. Ten patients with 1 to 7.6 years of follow-up after diagnosis showed no evidence of disease after radiation and/or chemotherapy. Like MZBCLs outside of the CNS, they consisted of CD20⁺, CD3^– small B lymphocytes with varying degrees of plasmacytic differentiation and predominantly light-chain restriction (78%). Lymphoid follicles with follicular colonization were seen in three patients and deposition of amyloid was noted in samples from two patients, one of which was tumefactive. Neither Bcl-6 protein nor Epstein-Barr virus–encoded RNA was expressed. Trisomy 3 was detected in six of 12 patients, with no rearrangements of MALT1 or IgH and no trisomies of 7, 12, or 18 detected.

CONCLUSION: Our data suggest that intracranial MZBCL is an indolent primary CNS lymphoma that typically presents as a meningioma-like dural-based mass. Trisomy 3, but not MALT1 or IgH translocation, is a common genetic abnormality that may contribute to the pathogenesis of this CNS lymphoma.

Authors' disclosures of potential conflicts of interest are found at the end of this article.

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