With no indication of systemic involvement, primary central nervous system lymphoma (PCNSL) is a rare extranodal lymphomatous cancer that affects the brain, spinal cord, leptomeninges, or vitreoretinal region. Because the clinical presentation of PCNSL differed according to the affected structures, a high level of suspicion was needed to make the diagnosis.

The start of therapy was crucial for optimum neurologic recovery and disease management. The invention and widespread use of high-dose methotrexate (MTX) chemotherapy, regarded as the cornerstone of first-line polychemotherapy treatment, greatly improved the prognosis of PCNSL over the last few decades. After MTX-based therapy is finished, a consolidation approach is frequently needed to extend the duration of response. Radiation therapy, maintenance therapy, nonmyeloablative chemotherapy, or myeloablative therapy followed by an autologous stem cell transplant can all be used as consolidation. Unfortunately, relapse happens often even after consolidation, and the 5-year survival rate is only between 30% and 40%. The activation of the B-cell receptor pathway, immune evasion, and a reduced tumor immunological microenvironment have all been linked to important pathways in tumor pathogenesis by new insights into the pathophysiology of PCNSL.

As a result of the discoveries, brand-new tiny compounds that target these abnormal pathways have been found. The Bruton tyrosine kinase inhibitor ibrutinib and immunomodulatory medications (lenalidomide or pomalidomide) have demonstrated encouraging clinical response rates for relapsed/refractory PCNSL and are being utilized more often to treat recurrent illness. The clinical presentation of PCNSL, the method for work-up and staging, and an overview of recent developments in the knowledge of the pathogenesis and current therapeutic approaches for immunocompetent patients were all included in the study.

Reference: ashpublications.org/blood/article/140/9/971/477460/Primary-central-nervous-system-lymphoma