[Primary central nervous system diffuse large B cell lymphoma: a clinicopathologic and molecular study]

Area of Science:

• Neurology
• Oncology
• Pathology

Background:

• Primary central nervous system diffuse large B-cell lymphoma (DLBCL) is a rare and aggressive non-Hodgkin lymphoma.
• Understanding its clinicopathologic features and molecular alterations is crucial for improving patient outcomes.

Purpose of the Study:

• To investigate the clinicopathologic characteristics, immunophenotype, and EB virus-related molecular genetic alterations in primary CNS DLBCL.
• To correlate these findings with clinical prognosis.

Main Methods:

• Retrospective analysis of 30 primary CNS DLBCL cases.
• Histological evaluation, immunophenotyping, in situ hybridization for EBER mRNA, and interphase fluorescence in situ hybridization (FISH) for bcl-2, bcl-6, and C-MYC gene abnormalities.

Main Results:

• The study identified distinct clinicopathologic patterns, including angiocentric growth and perivascular invasion.
• Germinal center B-cell-like (GCB) and non-GCB subtypes were identified, with varying frequencies of bcl-2, bcl-6, and C-MYC alterations.
• Median survival was 10 months, with a one-year survival rate of 46.7%, highlighting the aggressive nature of the disease.

Conclusions:

• Prognosis is influenced by age, performance status, IPI score, immune classification, and treatment.
• Primary CNS DLBCL exhibits rapid progression and high mortality.
• Surgical resection combined with high-dose methotrexate or cytarabine chemotherapy appears to be the optimal treatment strategy.