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23 - Central nervous system lymphoma

Published online by Cambridge University Press:  10 January 2011

By
Elizabeth R. Gerstner  and
Tracy T. Batchelor
Edited by
Susan O'Brien ,
Julie M. Vose  and
Hagop M. Kantarjian
Elizabeth R. Gerstner
Affiliation:
The Stephen E. and Catherine Pappas Center for Neuro-Oncology, Massachusetts General Hospital Cancer Center, Boston, MA, USA
Tracy T. Batchelor
Affiliation:
The Stephen E. and Catherine Pappas Center for Neuro-Oncology, Massachusetts General Hospital Cancer Center, Boston, MA, USA
Susan O'Brien
Affiliation:
University of Texas/MD Anderson Cancer Center, Houston
Julie M. Vose
Affiliation:
University of Nebraska Medical Center, Omaha
Hagop M. Kantarjian
Affiliation:
University of Texas/MD Anderson Cancer Center, Houston
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Summary

Introduction

Central nervous system (CNS) lymphoma may be isolated to the brain or leptomeninges in the absence of systemic disease (primary CNS lymphoma) or may occur as a neurologic complication of systemic lymphoma (secondary CNS lymphoma), usually in the form of leptomeningeal dissemination.

Primary CNS lymphoma

Primary central nervous system lymphoma (PCNSL) is an uncommon variant of extranodal non-Hodgkin lymphoma (NHL) that can affect multiple parts of the neuraxis including the eyes, brain, leptomeninges, or spinal cord. PCNSL accounts for approximately 3% of all the primary CNS tumors diagnosed each year in the United States and approximately 2–3% of all cases of NHL diagnosed each year. The incidence of PCNSL has increased over the past three decades, largely because of the human immunodeficiency virus (HIV) pandemic, but recent data suggest that the incidence has stabilized or is decreasing. Acquired or congenital immunodeficiency is the only established risk factor for PCNSL.

Since PCNSL is a rare malignancy, it has been challenging to study, which partially explains why it has been difficult to establish an effective standard of care for this type of lymphoma. In PCNSL, remission may be achieved for a few years but the tumor relapses in most cases. This chapter will focus on non-HIV-related CNS lymphoma since HIV-associated lymphomas are covered elsewhere.

Pathology and biology

Approximately 90% of non-HIV-associated PCNSL cases are diffuse large B-cell type with the remaining 10% consisting of poorly characterized low-grade lymphomas, Burkitt lymphomas, or T-cell lymphomas.

Type
Chapter
Information
Management of Hematologic Malignancies , pp. 449 - 461
Publisher: Cambridge University Press
Print publication year: 2010

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