Abstract Primary central nervous system lymphoma (PCNSL) is a rare aggressive B-cell non-Hodgkin lymphoma confined to the central nervous system, without systemic involvement. The incidence has increased over the past three decades. The prognosis has improved in patients up to 70 years old, and this type of lymphoma can be potentially cured. The gold standard of diagnosing PCNSL is histological, usually on a brain biopsy specimen via a neurosurgical procedure. Recent developments in both imaging and laboratory analyses of the cerebrospinal fluid, can be helpful in narrowing the differential diagnosis, diagnosing PCNSL itself, and in follow-up after treatment. This narrative review gives an overview of the epidemiology, diagnosis, and treatment of PCNSL, with an emphasis on recent developments in diagnostic techniques and treatment.
Meulen et al. (Mon,) studied this question.