A primary lymphoma of the central nervous system

In the absence of evidence 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 between the affected structures, a high level of suspicion was required to make the diagnosis.

The start of treatment was crucial for optimal neurological recovery and disease management. The invention and widespread use of high-dose methotrexate (MTX) chemotherapy, considered the cornerstone of first-line combination chemotherapy treatment, has greatly improved the prognosis of PCNSL in recent decades. After MTX therapy is completed, a consolidation approach is often needed to prolong the duration of the response. Radiation therapy, maintenance therapy, nonmyeloablative chemotherapy, or myeloablative therapy followed by autologous stem cell transplantation can all be used as consolidation. Unfortunately, relapses often occur even after consolidation, and the 5-year survival rate is only 30-40%. 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 through new insights into the pathophysiology of PCNSL.

As a result of these discoveries, whole new tiny compounds that target these abnormal pathways have been discovered. The Bruton tyrosine kinase inhibitor ibrutinib and immunomodulating drugs (lenalidomide or pomalidomide) have demonstrated encouraging clinical response rates for relapsed/refractory PCNSL and are being used more often to treat recurrent disease. The clinical presentation of the PCNSL, the workup and staging method, as well as an overview of recent developments in knowledge of 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

Comments are closed.