Primary CNS lymphoma: clinical presentation, pathological classification, molecular pathogenesis and treatment

Area of Science:

• Neuro-oncology
• Hematology
• Immunology

Background:

• Primary CNS lymphomas (PCNSL) are aggressive B-cell non-Hodgkin lymphomas originating within the central nervous system.
• PCNSL incidence is rising in both immunocompromised and immunocompetent individuals.
• Germinal center B cells are identified as the cellular origin, but molecular pathogenesis requires further elucidation.

Purpose of the Study:

• To review current treatment strategies for PCNSL.
• To evaluate the efficacy and toxicity of radiotherapy (RT) and chemotherapy, particularly high-dose methotrexate (HD-MTX).
• To explore the potential of chemotherapy-alone protocols for improved outcomes and quality of life.

Main Methods:

• Review of existing literature on PCNSL treatment.
• Analysis of survival rates and treatment response associated with RT and combination therapies.
• Assessment of long-term neurotoxicity in PCNSL patients.

Main Results:

• Radiotherapy (RT) alone provides tumor response but is not curative, with a median survival of approximately 1 year.
• Combination therapy of RT and high-dose methotrexate (HD-MTX) shows improved survival rates in uncontrolled studies.
• Long-term neurotoxicity is a significant concern with combination therapy, especially in older patients.

Conclusions:

• Chemotherapy-alone protocols, including HD-MTX, show promising results for both survival and quality of life in long-term survivors.
• Systematic evaluation of chemotherapy-alone strategies is favored to mitigate treatment-related neurotoxicity.
• Further research is needed to establish optimal treatment guidelines for PCNSL.