Lymphomas of Central Nervous System

Central nervous systemCentral nervous system (CNS) (CNS) lymphoma consists of primary central nervous system lymphomaPrimary Central Nervous System Lymphoma (PCNSL) (PCNSL) and secondary CNS involvement by systemic lymphoma. This chapter focuses on the former. PCNSL is a relative rare disease, accounting for approximately 2.4–4.9% of all primary CNS tumors. It is an extra-nodalExtra-nodal variant of non-Hodgkin's lymphoma (NHL)Non-Hodgkin's lymphoma (NHL), confined to the brain, leptomeningesLeptomeninges, spinal cordSpinal cord, and eyes, with no systemic involvement. Recently, elderly patients (≥ 60 years) are increasing. Histologically, B cell blasts, which originate from late germinal centerGerminal center exit B cell, are growing and homing in CNS. Immunohistochemically, these cells are positive for PAX5, CD19, CD20, CD22, and CD79a. PCNSLPrimary Central Nervous System Lymphoma (PCNSL) shows relatively characteristic appearances on CT, MR imaging, and PETPositron Emission Tomography (PET). Treatment first line of PCNSLPrimary Central Nervous System Lymphoma (PCNSL) is HD-MTX-based chemotherapyChemotherapy with or without rituximabRituximab and irradiationIrradiation. Severe side-effect of this treatment is delayed onset neurotoxicityNeurotoxicity, which cause of cognitive impairmentCognitive impairment. Therefore, combined chemotherapyChemotherapy alone or chemotherapyChemotherapy with reduced-dose irradiationIrradiation is more recommended for elderly patients. There is no established standard care for relapse of the PCNSLs. TemsirolimusTemsirolimus, lenalidomideLenalidomide, temozolomide, and Bruton's tyrosine kinaseBruton's tyrosine kinase (BTK) (BTK) inhibitor ibrutinibIbrutinib are candidates for refractory patients. The prognosisPrognosis of PCNSLPrimary Central Nervous System Lymphoma (PCNSL) has significantly improved over the last decades (median OS: 26 months, 5-year survivalSurvival: 31%). Younger than 60 age and WHOWorld Health Organization (WHO) performance status less than < or = 1 are associated with a significantly better overall survivalSurvival.