Primary Central Nervous System Lymphoma-PART 1: Epidemiology, Diagnosis, Staging, and Prognosis

Primary central nervous system (CNS) lymphoma is a rare CNS neoplasm. Its highest incidence is in the elderly and the immunocompromised. The initial steps in establishing a diagnosis involve CNS imaging. Familiarity with the clinical presentation is important in order to limit the risk of a nondiagnostic biopsy. In addition to confirming the diagnosis, it is wise to evaluate for extra-CNS disease. There are important differences in the presentation and evaluation of immunocompetent patients and those of immunocompromised patients; we will delineate these in this review. Appropriate initial clinical evaluations facilitate optimal therapeutic management for patients with primary CNS lymphoma. This is of particular importance because primary CNS lymphoma is a potentially curable disease, despite the high likelihood of recurrence.