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Published on: January 12, 2024
[Retrospective study of 48 cases of primary central nervous system lymphoma]
Lucas Alessandro1, José M Pastor Rueda1, Juan F Villalonga2
1Servicio de Neurología, Instituto de Investigaciones Neurológicas Raúl Carrea (FLENI), Buenos Aires, Argentina.
Insights
Primary central nervous system lymphoma (PCNSL) primarily affects immunocompetent elderly males. Early diagnosis of PCNSL is crucial, as clinical and MRI findings are variable.
Area of Science:
- Neurology
- Oncology
- Immunology
Background:
- Primary central nervous system lymphoma (PCNSL) is a rare non-Hodgkin lymphoma confined to the CNS.
- Diffuse large B-cell lymphoma (DLBCL) constitutes the majority of PCNSL cases, predominantly affecting individuals aged 50-70.
- Immunodeficiency is a significant risk factor, though many PCNSL patients are immunocompetent.
Purpose of the Study:
- To evaluate the immune status of PCNSL patients.
- To analyze the clinical presentation and complementary study findings in PCNSL.
- To identify key diagnostic indicators for PCNSL.
Main Methods:
- Retrospective analysis of 48 PCNSL cases diagnosed between January 1992 and May 2015.
- Review of patient immune status, clinical manifestations, brain MRI, and cerebrospinal fluid (CSF) analysis.
- Histopathological classification of lymphoma subtypes.
Main Results:
- The median age at diagnosis was 61 years, with a male predominance (2.1:1).
- 85% of patients were immunocompetent. Brain MRI revealed parenchymal involvement in 94% of cases.
- Diffuse large B-cell lymphoma (83%) was the most frequent type, with B-cell origin in 96%.
Conclusions:
- PCNSL in this series was more prevalent in immunocompetent, elderly males.
- Clinical and MRI findings at initial evaluation were diverse.
- Prompt clinical suspicion is essential for early PCNSL diagnosis and to prevent delayed or inaccurate treatment.
Abstract:
Primary central nervous system lymphoma (PCNSL) is an infrequent form of non-Hodgkin lymphoma restricted to the CNS. More than 90% are type B and mainly affect patients aged 50-70 years. Immunodeficiency is the most important risk factor. The aim of our study was to evaluate the immune status, clinical presentation and findings in complementary studies of PCNSL patients. A retrospective analysis of 48 cases treated in our center between January 1992 and May 2015 was performed. Median age at diagnosis was 61 years (range 25-84); with male predominance (2.1:1). Forty one cases (85%) were immunocompetent patients. Brain MRI findings showed parenchymal involvement in 45 cases (94%), 43% with frontal lobe and 35% basal ganglia, 4% had meningeal involvement and 2% had ophthalmic involvement at diagnosis. Fifty-five percent had restricted signal on diffusion weighted imaging and contrast enhancement was found in 89%. Pyramidal syndrome was the main initial clinical manifestation (56%). There were abnormal findings in 62% of CSF samples, but in only 11.1% positive cytology results were detected. The most frequent type was diffuse large B-cell lymphoma (83%), being B-cell type the most common form between them (96%). In our series PCNSL was more frequent in immunocompetent elderly male subjects. At initial evaluation, clinical manifestations and MRI findings were variable. The initial suspicion of this entity would allow an early diagnosis, avoiding empirical treatments that may confuse or delay diagnosis.
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