Primary central nervous system lymphoma - an overview

Introduction. Primary central nervous system lymphoma is a rare entity mostly presenting with non-GCB diffuse large B-cell lymphoma, being confined to the brain, spinal cord, meninges, and eyes. Diagnosis. The diagnosis frequently established by stereotactic or open brain biopsy, but in some cases isolated leptomeningeal involvement, only way identify atypical/monoclonal lymphocytes cerebrospinal fluid. By workup, we aim define extent of disease exclude systemic involvement. Treatment. Treatment tailored according patient?s age, fitness, vital organ function, comorbidities, available therapy. backbone induction treatment high-dose methotrexate, usually within polychemotherapy. Consolidation phase matter debate between two approaches: 1. high dose chemotherapy autologous stem cell transplantation, which appears be preferable option for young fit patients, 2. whole radiotherapy, preserved transplant-ineligible ones. Whole radiotherapy has been raising concerns because frequent cognitive impairment, significantly diminished reducing irradiation dose. Despite comprehensive approach, many patients relapse, since prognosis relapsed/refractory devastating, there sense urgency novel strategies. Several targeted agents immunomodulatory drugs have investigated settings both initial therapy, limited success. Ibrutinib monotherapy can induce durable remissions first line, relapse/refractory settings, results are controversial. Conclusion. Adequate patient selection new prospective trials should improve survival preserve neurological status.