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Published on: November 17, 2020
Primary central nervous system lymphoma
Sharathkumar Bhagavathi1, Jon D Wilson
1Department of Anatomic Pathology, William Beaumont Hospital, Royal Oak, MI 48076, USA. vmsharathkumar@yahoo.com
Insights
Primary central nervous system lymphoma (PCNSL) is a rare non-Hodgkin lymphoma affecting the brain. While its incidence is rising, PCNSL has a poorer prognosis than other lymphomas and requires combined treatment.
Area of Science:
- Neurology
- Oncology
- Pathology
Background:
- Primary central nervous system lymphoma (PCNSL) is an uncommon extranodal non-Hodgkin lymphoma with increasing incidence.
- It affects both immunocompromised and immunocompetent individuals, often presenting with increased intracranial pressure and focal neurological deficits.
Purpose of the Study:
- To summarize the key characteristics, diagnosis, and treatment of primary central nervous system lymphoma.
- To highlight the challenges in understanding PCNSL pathogenesis and its differential diagnosis.
Main Methods:
- Review of existing literature on PCNSL.
- Analysis of clinical presentation, histopathology, and immunohistochemistry findings.
- Discussion of treatment modalities and prognostic factors.
Main Results:
- PCNSL most commonly presents as solitary, periventricular lesions and is typically a diffuse large B-cell lymphoma.
- Characteristic angiocentric morphology and B-cell marker positivity aid diagnosis.
- Differential diagnoses include gliomas, metastases, and infectious lesions.
Conclusions:
- PCNSL is an aggressive brain lymphoma with a worse prognosis than systemic lymphomas.
- Combined radiotherapy and chemotherapy are standard treatments.
- Further research into molecular mechanisms and biomarkers is needed.
Abstract:
Primary central nervous system lymphoma (PCNSL) is an uncommon extranodal non-Hodgkin lymphoma. Its incidence has increased during the last 3 decades and has been reported in both immunocompromised and immunocompetent patients. Immunocompromised patients are affected at a younger age compared with immunocompetent patients. It presents with raised intracranial pressure and focal neurologic and neuropsychiatric symptoms. The lesions are typically solitary. The majority of the lesions are located in the periventricular area, whereas in a few cases they are located in the supratentorial area. Diffuse large B-cell lymphomas constitute most PCNSLs, whereas T-cell, low-grade, anaplastic, and Hodgkin lymphomas are rarely encountered. The morphology of PCNSL shows a characteristic angiocentric pattern and is positive for B-cell markers by immunohistochemistry. The differential diagnosis of PCNSL includes central nervous system gliomas, metastatic tumors, demyelinating disorders, subacute infarcts, and space-occupying lesions due to an infectious etiology. The understanding of the molecular mechanisms involved in the pathogenesis of PCNSL and the identification of molecular biomarkers have lagged behind that of systemic nodal lymphomas. Primary central nervous system lymphomas are treated with combined radiotherapies and chemotherapies. The prognosis for PCNSL is worse than for other extranodal lymphomas.
