Primary central nervous system lymphoma: A curable disease

Primary central nervous system lymphoma is a rare subtype of non‐Hodgkin lymphoma that is confined to the brain, leptomeninges, or the eye and is associated with a relatively poor prognosis compared to other extranodal diffuse large B‐cell lymphomas. However, methotrexate‐based induction chemotherapy followed by consolidative chemotherapy or high‐dose therapy and autologous stem cell transplantation is associated with improved survival and reduced neurotoxicity. Aberrant activation of B‐cell receptor signaling and activation of nuclear factor kappa beta is a frequent genetic alteration and offers opportunities for targeted therapies in this lymphoma subtype.