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Clinical presentation, pathologic features, and diagnosis of primary central nervous system lymphoma

Authors
Tracy Batchelor, MD, MPH
Jay S Loeffler, MD
Section Editor
Arnold S Freedman, MD
Deputy Editor
April F Eichler, MD, MPH

INTRODUCTION

Primary central nervous system lymphoma (PCNSL) is an uncommon variant of extranodal non-Hodgkin lymphoma (NHL) that involves the brain, leptomeninges, eyes, or spinal cord without evidence of systemic disease.

The epidemiology and clinical presentation vary depending upon the immunocompetence of the patient. This topic will discuss the clinical presentation, pathologic features, and diagnosis of PCNSL in immunocompetent patients. AIDS-related PCNSL is discussed separately. (See "AIDS-related lymphomas: Primary central nervous system lymphoma".)

Lymphomatous involvement of the central nervous system in patients with widespread systemic nodal or extra-nodal lymphoma is also reviewed separately. (See "Treatment and prognosis of primary central nervous system lymphoma" and "Clinical presentation and diagnosis of secondary central nervous system lymphoma".)

EPIDEMIOLOGY

PCNSL represents approximately 4 percent of newly diagnosed primary central nervous system (CNS) tumors, with an age-adjusted incidence rate of four cases per million persons per year [1,2]. The incidence in the general population rose from the 1960s to the 1990s, peaked in the mid-1990s, and then declined [1,3-13]. Such changes were largely driven by PCNSL cases in men between the ages of 20 and 64 years [14]. Thus, the trend has been attributed in large part to changes in HIV/AIDS incidence and management over the same time period. In contrast, the incidence rate in adults >65 years of age has steadily risen, even in the last decade [14].

Most cases of non-AIDS related PCNSL are diagnosed in patients between 45 and 65 years of age, with a median age at diagnosis in the fifth decade [3,15-17]. Rare cases have been described in children with a median age of 14 years [4,18]. Men and women are equally affected.

                 

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Literature review current through: Nov 2016. | This topic last updated: Wed Nov 25 00:00:00 GMT+00:00 2015.
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