Lymphomas of Central Nervous System

Area of Science:

• Oncology
• Neurology
• Hematology

Background:

• Primary central nervous system lymphoma (PCNSL) is a rare extranodal non-Hodgkin's lymphoma (NHL) confined to the CNS.
• It accounts for approximately 2.4-4.9% of primary CNS tumors, with a recent increase in elderly patients.
• Histologically, PCNSL involves B cell blasts positive for specific markers like PAX5, CD19, and CD20.

Purpose of the Study:

• To provide an overview of primary central nervous system lymphoma (PCNSL).
• To discuss diagnostic features, treatment strategies, and prognostic factors for PCNSL.
• To highlight recent advancements and challenges in managing PCNSL.

Main Methods:

• Review of current literature on PCNSL diagnosis and treatment.
• Analysis of characteristic imaging findings on CT, MR imaging, and PET scans.
• Summary of standard and emerging therapeutic approaches, including chemotherapy and targeted therapies.

Main Results:

• PCNSL exhibits characteristic imaging appearances.
• HD-MTX-based chemotherapy with or without rituximab and irradiation is a first-line treatment.
• Combined chemotherapy or reduced-dose irradiation is recommended for elderly patients due to neurotoxicity risks.

Conclusions:

• The prognosis for PCNSL has improved, with a median overall survival of 26 months and a 5-year survival rate of 31%.
• Younger age (<60) and good WHO performance status (< or = 1) are associated with better survival.
• Further research is needed for established standard care for relapsed PCNSL, with agents like ibrutinib showing promise.