Primary central nervous system lymphoma: epidemiology and clinical presentation

Abstract

Primary central nervous system lymphoma (PCNSL) is a non-Hodgkin lymphoma that can affect any component of the central nervous system: brain, eyes and/or spinal cord. While it is a rare entity, the incidence has been rising since the 1960s with an increase in incidence in the 1990s that coincided with the human immunodeficiency virus (HIV) pandemic. More recently in the last two decades, incidence has been rising in the elderly population. PCNSL can have a wide range of presentations given possible involvement of any part of the nervous system, which can often lead to a delay in the diagnosis and treatment. Depending on the location involved, PCNSL can present with a variety of symptoms. Intracranial lesions are the most common manifestation of PCNSL and the majority present with focal neurologic symptoms, but nonspecific non-specific neuropsychiatric symptoms are also common. Primary leptomeningeal involvement is rare and can manifest with cranial neuropathies. Primary vitreoretinal lymphoma can present with blurred vision and symptoms mimicking uveitis. Spinal cord involvement can present with subacute myelopathy and peripheral nerve involvement or neurolymphomatosis can present with focal sensory and motor involvement. Given the wide range of clinical presentation, an understanding of the variable clinical manifestations of PCNSL is important for prompt diagnosis and treatment.