An Update on the Diagnosis and Treatment for Primary CNS Lymphoma

Abstract

  The incidence of primary central nervous system lymphoma (PCNSL) has grown recently, and neurosurgeons in Japan frequently have the opportunity to treat it. PCNSL causes cognitive impairment and has several atypical imaging findings, making it challenging to diagnose. In cases where biopsy is challenging, certain biomarkers in cerebrospinal fluid and deoxyribonucleic acid and ribonucleic acid assays via liquid biopsy have recently been reported to be significant. Rituximab, methotrexate (MTX), procarbazine, and vincristine are increasing accepted as routine therapies in Japan, and MTX-based multidrug chemotherapy is recommended as induction therapy. High-dose cytarabine is employed for subsequent consolidation therapy. High-dose whole-brain radiation therapy should be avoided as much as possible during initial therapy due to neurotoxicity, such as cognitive impairment. MTX re-challenge is effective at recurrence ; however, the time until recurrence should be considered. Recently developed Bruton's tyrosine kinase inhibitors have attracted attention and show high response rates. However, there are issues with persistence and adverse events ; therefore, further research is required its use.