Primary central nervous system lymphoma pathophysiology
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Primary central nervous system lymphoma Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
Pathogenesis
Associated Conditions
- Primary central nervous system lymphoma is highly associated with Epstein-Barr virus (EBV) infection (> 90%) in immunodeficient patients (such as those with AIDS and iatrogenically immunosuppressed).[1]
Gross Pathology
- Primary central nervous system lymphoma presents as a solitary or multiple, well-defined or infiltrating mass lesion/s that can arise in the cortex, white matter, or deep grey matter (more common in low-grade lesions).[2]
- The lesion(s) are mainly located at supratentorial level, usually in the periventricular regions, infiltrating the corpus callosum and the basal ganglia.[3]
- They may demonstrate areas of necrosis, especially in immunodeficient patients.
- Origin of malignant cells is not well understood as intra-axial CNS does not have lymphatic system.[2]
Gallery
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![Gross pathological specimen primary central nervous system lymphoma in the brain parenchyma. This autopsy photograph shows involvement of cerebral hemispheres by a primary CNS lymphoma in an AIDS patient. It was a large B-cell lymphoma and the patient succumbed to it within 6 months of diagnosis.[4]](/images/9/9f/Gross_pathological_image_of_primary_central_nervous_system_lymphoma_image_1.jpg)
Gross pathological specimen primary central nervous system lymphoma in the brain parenchyma. This autopsy photograph shows involvement of cerebral hemispheres by a primary CNS lymphoma in an AIDS patient. It was a large B-cell lymphoma and the patient succumbed to it within 6 months of diagnosis.[4]
![Gross pathological specimen primary central nervous system lymphoma in the brain parenchyma. This autopsy photograph shows involvement of cerebral hemispheres by a primary CNS lymphoma in an AIDS patient. It was a large B-cell lymphoma and the patient succumbed to it within 6 months of diagnosis.[4]](/index.php/File:Gross_pathological_image_of_primary_central_nervous_system_lymphoma_image_1.jpg)
Microscopic Pathology
- The vast majority (>90%) of primary central nervous system lymphoma are B-cell in origin: diffuse large B-cell lymphoma and high-grade Burkitt-like B-cell lymphoma.[2]
- Malignant cells tend to accumulate around blood vessels.
- Low-grade tumors are more frequently T-cell in origin.[2]
- On microscopic histopathological analysis, primary central nervous system lymphoma is characterized by a perivascular distribution composed of large cells with prominent nucleoli.[1]
Gallery
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![Micrograph from a brain biopsy demonstrating a primary CNS lymphoma with the characteristic perivascular distribution composed of large cells with prominent nucleoli, on HPS stain.[1]](/images/d/d1/Microscopic_pathological_image_of_primary_central_nervous_system_lymphoma_image_1.jpg)
Micrograph from a brain biopsy demonstrating a primary CNS lymphoma with the characteristic perivascular distribution composed of large cells with prominent nucleoli, on HPS stain.[1]
![Micrograph from a brain biopsy demonstrating a primary CNS lymphoma with the characteristic perivascular distribution composed of large cells with prominent nucleoli, on HPS stain.[1]](/index.php/File:Microscopic_pathological_image_of_primary_central_nervous_system_lymphoma_image_1.jpg)
References
- ↑ 1.0 1.1 1.2 Primary central nervous system lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Primary_central_nervous_system_lymphoma. Accessed on February 18, 2016
- ↑ 2.0 2.1 2.2 2.3 Pathology of primary central system lymphoma. Dr Amir Rezaee and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/primary-cns-lymphoma. Accessed on February 18, 2016
- ↑ Manenti, G.; Di Giuliano, F.; Bindi, A.; Liberto, V.; Funel, V.; Garaci, F. G.; Floris, R.; Simonetti, G. (2013). "A Case of Primary T-Cell Central Nervous System Lymphoma: MR Imaging and MR Spectroscopy Assessment". Case Reports in Radiology. 2013: 1–5. doi:10.1155/2013/916348. ISSN 2090-6862.
- ↑ Image courtesy of Dr. A.Prof Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC