Primary CNS Lymphomas: Diagnosis and Treatment

Primary central nervous system lymphoma (PCNSL) is a highly aggressive non-Hodgkin lymphoma confined to the CNS, including the brain, spine, the meninges, and eyes. The B-cell receptor pathway, with its downstream effector NFĸB, is affected by frequent mutations, mainly in MYD88 and CD79B. Advanced imaging and non-invasive diagnostic analyses of cerebrospinal fluid (CSF) may aid in discriminating PCNSL from other brain lesions. Yet, histopathologic diagnosis of tumor tissue gained by (stereotactic) biopsy still is standard. High-dose systemic methotrexate (HDMTX) is the backbone of successful therapy, often applied in combination with cytarabine (araC), rituximab, and other agents. Induction therapy is followed by consolidation with high-dose chemotherapy followed by autologous stem cell transplantation (HDASCT), by intensified conventional chemotherapy and/or by (dose-reduced) whole-brain radiotherapy (WBRT). While the majority of patients initially respond, more than half will relapse or show primary progression. Thus, novel agents such as e.g., the Bruton tyrosine kinase inhibitor ibrutinib, immunomodulatory drugs such as lenalidomide or checkpoint inhibitors are investigated within clinical trials.

This Special Issue highlights recent findings on the molecular pathogenesis of PCNSL, novel developments of molecular-based non-invasive diagnostics, the backbone of current therapy in clinical practice, the therapeutic potential of novel agents and CAR-T cells, and last but not least, factors influencing Quality of Life in long-term survivors.

Prof. Dr. Uwe Schlegel
Guest Editor

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