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13 - Central nervous system lymphomas
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- By Andrés J. M. Ferreri, San Raffaele Scientific Institute, Lisa M. DeAngelis, Memorial Sloan-Kettering Cancer Center
- Edited by Robert Marcus, King's College London, John W. Sweetenham, University of Utah, Michael E. Williams
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- Book:
- Lymphoma
- Published online:
- 18 December 2013
- Print publication:
- 05 December 2013, pp 208-225
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Summary
A variety of lymphomas can involve the central nervous system (CNS) at different phases of their evolution, both in immunocompetent and immunocompromised individuals. They represent a heterogeneous group of malignancies, with variable clinical and behavioral characteristics, and require different therapeutic approaches. In this chapter, the therapeutic management of these malignancies will be analyzed separately in three main entities: primary CNS lymphomas (PCNSL), secondary CNS lymphomas (SCNSL), and other, less common, forms of CNS lymphomas.
Histopathology
Primary diffuse large B-cell lymphoma (DLBCL) of the CNS shows diffuse parenchymal growth with dense cuffing in perivascular spaces. The latter is best appreciated towards the periphery of the tumor. Large areas of necrosis may be present, especially in those treated with steroids prior to biopsy. Towards the periphery of the tumor the neoplastic cells are often admixed with astrocyte gliosis. Cytologically, the neoplastic cells resemble those of DLBCL encountered elsewhere. Similarly, the immunophenotype of primary CNS DLBCL is similar to those outside the CNS and is positive for B-cell markers (CD20, CD79a, and PAX-5). Strong IRF4/MUM1 staining is seen in 90% of cases. CD10 is expressed by a minority of cases, while many express BCL-6. BCL-2 is often expressed. As these lymphomas (by definition) occur in immunocompetent patients, Epstein–Barr virus (EBV) is generally absent.
Many lymphomas involving the CNS in immunocompromised patients are EBV-positive and show features of DLBCL, many with an immunoblastic morphology. Burkitt lymphoma, a common lymphoma in the immunocompromised patient, may involve the CNS.
14 - Central nervous system lymphoma
- from Part II - LYMPHOMA SUBTYPES
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- By Andrés J. M. Ferreri, Medical Oncology Unit, Department of Oncology, San Raffaele H. Scientific Institute Via Olgettina 60, 20132, Milan, Italy, Lisa M. DeAngelis, Department of Neurology, Memorial Sloan-Kettering Cancer Center, 1275, York Avenue, New York, NY, 10021, USA, Andrew Wotherspoon, Department of Histopathology, Royal Marsden Hospital, Fulham Road, London, SW3 6JJ, UK, Andreas Rosenwald, Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany, German Ott, Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany
- Edited by Robert Marcus, John W. Sweetenham, Case Western Reserve University, Ohio, Michael E. Williams, University of Virginia
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- Book:
- Lymphoma: Pathology, Diagnosis and Treatment
- Published online:
- 05 March 2010
- Print publication:
- 21 June 2007, pp 200-214
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- Chapter
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Summary
INTRODUCTION
A variety of lymphomas can involve the central nervous system (CNS), at different phases of their evolution, in both immunocompetent and immunocompromised individuals. They represent a heterogeneous group of malignancies, with variable clinical and behavioral characteristics, requiring different therapeutic approaches. In this chapter, the therapeutic management of these malignancies will be analyzed separately in three main entities: primary CNS lymphomas (PCNSL), secondary CNS lymphomas (SCNSL) and other, less common, forms of CNS lymphomas.
PATHOLOGY
The vast majority of CNS lymphomas are diffuse large B-cell lymphomas (DLBCL) that share the morphological and immunophenotypic characteristics similar to those of DLBCLs encountered elsewhere. They may show a perivascular growth pattern. The perivascular infiltrate is associated with increased reticulin fibres and the periphery of areas of involvement frequently shows astrocyte gliosis. Many immunocompromised patients show features similar to Burkitt's lymphoma, while others show a more immunoblastic morphology.
Rare cases of small lymphocytic, lymphoplasmacytic and T-cell lymphoma similar to those seen in tissue outside the CNS have been described. Secondary involvement by lymphoma originating elsewhere is also encountered.
Immunophenotypically CNS lymphomas recapitulate the staining pattern of similar lymphomas encountered outside the CNS. The DLBCLs are positive for CD20 and CD79a with expression of bcl-2 protein. A proportion express CD10 and bcl-6, but they are usually negative for CD5 and CD23. Large B-cell lymphomas in immunocompromised patients frequently contain Epstein–Barr virus (EBV).