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 Patrick; Yachnis, Anthony T.; Burger, Peter C.; Scheithauer, Bernd W.; Perry, Arie

doi:10.1200/jco.2005.17.624

Cited by 146 publications

(171 citation statements)

References 44 publications

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“…28 The MZBCLs primary to the CNS, although very rare, are considered by some to be the most common low-grade PCNSL. 41 They differ from the other types of PCNSL discussed so far in that they typically are dura-based and mimic meningiomas. They are also notable for their favorable prognosis and higher incidence in women.…”

Section: Marginal Zone B-cell Lymphomas Of the Dura Matermentioning

confidence: 99%

“…41 The tumors involved mainly the dura mater and leptomeninges, with occasional instances of extension down Virchow-Robin spaces. Like MZBCLs elsewhere, the neoplastic lymphocytes were small to medium and had moderate amounts of cytoplasm and irregular nuclei, resembling follicular small-cleaved cells.…”

Section: Marginal Zone B-cell Lymphomas Of the Dura Matermentioning

confidence: 99%

“…41 It has subsequently been divided in the WHO classification scheme into three subtypes: nodal, extranodal (mucosaassociated lymphoid tissue), and splenic, based on differences in clinical behavior. All types are believed to be derived from postgerminal center marginal zone B-cells that surround the lymphoid follicle, often referred to as monocytoid B cells because of their abundant clear cytoplasm and distinct cytoplasmic borders.…”

Section: Marginal Zone B-cell Lymphomas Of the Dura Matermentioning

confidence: 99%

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Pathology of primary central nervous system lymphoma

Commins¹

2006

FOC

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✓ The understanding of primary central nervous system lymphoma (PCNSL) has lagged behind that of the much more common systemic, nodal lymphomas. Reasons for this include the relative rarity of PCNSL and the fact that these lesions differ in some ways from their histologically similar systemic counterparts. The purpose of this paper is twofold: first, the author provides concise descriptions of the pathological features of both common and uncommon types of PCNSL while discussing issues such as the confusion surrounding histological classification of PCNSLs, problems related to intraoperative diagnosis, and the appropriate diagnostic work up. Second, the author discusses a small number of molecular studies to demonstrate the great promise they offer. Such studies have already clarified some issues, including the category of lymphocyte from which the majority of PCNSLs are derived. Hopefully in the future these studies will help guide treatment decisions.

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Section: Marginal Zone B-cell Lymphomas Of the Dura Matermentioning

confidence: 99%

Section: Marginal Zone B-cell Lymphomas Of the Dura Matermentioning

confidence: 99%

Section: Marginal Zone B-cell Lymphomas Of the Dura Matermentioning

confidence: 99%

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Pathology of primary central nervous system lymphoma

Commins¹

2006

FOC

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“…Because they are morphologically indistinguishable from systemic DLBCLs, the World Health Organization classification of tumors of hematopoietic and lymphoid tissues does not recognize PCNSL as a separate entity [4]. The remaining cases of PCNSL are T-cell lymphomas (2%-5%) [5] or, in rare instances, low-grade B-cell lymphomas of the lymphoplasmocytic (Waldenström macroglobulinemia), follicular, or mucosa-associated lymphoid tissue type [6]. Little is known about the tumorigenesis of PCNSL.…”

Section: Pathology and Pathogenesismentioning

confidence: 99%

Primary CNS Lymphoma in Immunocompetent Patients

et al. 2009

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Primary central nervous system lymphoma (PCNSL) constitutes a rare group of extranodal non-Hodgkin's lymphomas (NHLs), primarily of B cell origin, whose incidence has markedly increased in the last three decades. Immunodeficiency is the main risk factor, but the large majority of patients are immunocompetent. Recent evidence suggests a specific tumorigenesis that may explain their particular clinical behavior compared with systemic NHL. The addition of i.v. high-dose methotrexate (MTX) chemotherapy to whole-brain radiotherapy (WBRT) has considerably improved the prognosis, leading to a threefold longer median survival time compared with WBRT alone and represents the current standard of care. However, this combined treatment exposes the patient, especially the elderly, to a high risk for delayed neurotoxicity. In the older population (>60 years), there is growing evidence that MTX-based chemotherapy alone as initial treatment is the best approach to achieve effective tumor control without compromising patient quality of life. In the younger population, the risk for neurotoxicity is much lower, and this strategy is controversial because it may be associated with higher relapse rates. Future efforts should focus on the development of new polychemotherapy regimens allowing the reduction or deferral of WBRT in order to minimize the risk for delayed neurotoxicity. In this setting, intensive chemotherapy with autologous blood stem cell transplantation was recently demonstrated to be feasible and efficient as salvage therapy and is currently being evaluated as part of primary treatment. This review highlights the recent advances in the pathogenesis and treatment of PCNSL in the immunocompetent population.

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“…Since the initial description in 1997 by Kumar et al, 56 cases of primary meningeal MALT-NHL (PMML) have been reported so far [1][2][3][4][5], mostly under the form of case reports [2][3][4][5], but one large study [1]. Review of literature shows that PMML has somewhat common clinical characteristics and there are not well-established therapeutic guidelines.…”

Section: Primary Intracranial Meningeal Marginal Zone B Cell Lymphomamentioning

confidence: 99%

Primary intracranial meningeal marginal zone B cell lymphoma of malt type (PMML) with osseous infiltration

Famoso¹,

Ponzoni²,

Terreni³

et al. 2008

Ann Hematol

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

Abstract: 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.

Cited by 146 publications

References 44 publications

Pathology of primary central nervous system lymphoma

Pathology of primary central nervous system lymphoma

Primary CNS Lymphoma in Immunocompetent Patients

Primary intracranial meningeal marginal zone B cell lymphoma of malt type (PMML) with osseous infiltration

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