Medicinski pregled 2022 Volume 75, Issue Suppl. 1, Pages: 73-81
https://doi.org/10.2298/MPNS22S1073V
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Primary central nervous system lymphoma - an overview
Vuković Vojin 
(University Clinical Center of Serbia, Belgrade, Clinic of Hematology + University of Belgrade, Faculty of Medicine, Belgrade), vojin.vukovic@med.bg.ac.rs
Antić Darko (University Clinical Center of Serbia, Belgrade, Clinic of Hematology + University of Belgrade, Faculty of Medicine, Belgrade)
Otašević Vladimir (University Clinical Center of Serbia, Belgrade, Clinic of Hematology)
Sabljić Nikica (University Clinical Center of Serbia, Belgrade, Clinic of Hematology)
Šarac Sofija (University Clinical Center of Serbia, Belgrade, Clinic of Hematology)
Mihaljević Biljana (University Clinical Center of Serbia, Belgrade, Clinic of Hematology + University of Belgrade, Faculty of Medicine, Belgrade)
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.
Keywords: Central Nervous System Neoplasms, Lymphoma, Signs and Symptoms, Diagnosis, Therapeutics, Methotrexate, Morphological and Microscopic Findings, Positron Emission Tomography Computed Tomography, Magnetic Resonance Imaging
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