Clinicopathologic and genetic profile of intracranial marginal zone lymphoma

A primary low-grade CNS lymphoma that mimics meningioma

Pang Hsien Tu, Caterina Giannini, Alexander R. Judkins, Jason M. Schwalb, Richard Burack, Brian P. O'Neill, Anthony T. Yachnis, Peter C. Burger, Bernd W. Scheithauer, Arie Perry

Research output: Contribution to journalArticle

Abstract

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 κ 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.

Original languageEnglish (US)
Pages (from-to)5718-5727
Number of pages10
JournalJournal of Clinical Oncology
Volume23
Issue number24
DOIs
StatePublished - 2005

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Meningioma
Non-Hodgkin's Lymphoma
Lymphoma
Trisomy
Marginal Zone B-Cell Lymphoma
Centromere
Fluorescence In Situ Hybridization
Human Herpesvirus 4
Amyloid
B-Lymphocytes
RNA
Radiation
Light
Drug Therapy
Neoplasms
Proteins

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Clinicopathologic and genetic profile of intracranial marginal zone lymphoma : A primary low-grade CNS lymphoma that mimics meningioma. / Tu, Pang Hsien; Giannini, Caterina; Judkins, Alexander R.; Schwalb, Jason M.; Burack, Richard; O'Neill, Brian P.; Yachnis, Anthony T.; Burger, Peter C.; Scheithauer, Bernd W.; Perry, Arie.

In: Journal of Clinical Oncology, Vol. 23, No. 24, 2005, p. 5718-5727.

Research output: Contribution to journalArticle

Tu, PH, Giannini, C, Judkins, AR, Schwalb, JM, Burack, R, O'Neill, BP, Yachnis, AT, Burger, PC, Scheithauer, BW & Perry, A 2005, 'Clinicopathologic and genetic profile of intracranial marginal zone lymphoma: A primary low-grade CNS lymphoma that mimics meningioma', Journal of Clinical Oncology, vol. 23, no. 24, pp. 5718-5727. https://doi.org/10.1200/JCO.2005.17.624
Tu, Pang Hsien ; Giannini, Caterina ; Judkins, Alexander R. ; Schwalb, Jason M. ; Burack, Richard ; O'Neill, Brian P. ; Yachnis, Anthony T. ; Burger, Peter C. ; Scheithauer, Bernd W. ; Perry, Arie. / Clinicopathologic and genetic profile of intracranial marginal zone lymphoma : A primary low-grade CNS lymphoma that mimics meningioma. In: Journal of Clinical Oncology. 2005 ; Vol. 23, No. 24. pp. 5718-5727.
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abstract = "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 κ 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.",
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T2 - A primary low-grade CNS lymphoma that mimics meningioma

AU - Tu, Pang Hsien

AU - Giannini, Caterina

AU - Judkins, Alexander R.

AU - Schwalb, Jason M.

AU - Burack, Richard

AU - O'Neill, Brian P.

AU - Yachnis, Anthony T.

AU - Burger, Peter C.

AU - Scheithauer, Bernd W.

AU - Perry, Arie

PY - 2005

Y1 - 2005

N2 - 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 κ 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.

AB - 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 κ 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.

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