Abstract

Primary central nervous system lymphoma (PCNSL) is a rare extranodal aggressive non-Hodgkin lymphoma (NHL) that differs from other NHL in terms of morbidity, diagnosis, and treatment. PCNSL accounts for 1%-6% of all central nervous system tumors, and 4%-6% of extranodal lymphomas. The incidence of PCNSL has increased gradually in the past 10 years, from 0.15/100 000 to 0.48/100 000. The pathological diagnosis is the only method for definite diagnosis. Stereotactic puncture is the currently preferred invasive procedure. Magnetic resonance imaging is the gold standard for diagnosis and follow-up of PCNSL. The chemotherapy based on large doses of methotrexate (≥3 g/m2) is currently the main treatment. For patients who are suitable for autologous hematopoietic stem cell transplantation, autologous transplantation can improve the short-term and long-term survival. New drug treatment and clinical research are encouraged for patients with relapsed and refractory PCNSL. This review will briefly introduce the current status and progress in treatment of PCNSL. Key words: Lymphoma; Central nervous system; Drug therapy, combination

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