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