Primary central nervous system lymphoma pathophysiology
Primary central nervous system lymphoma Microchapters |
Differentiating Primary Central Nervous System Lymphoma from other Diseases |
---|
Diagnosis |
Treatment |
Case Studies |
Primary central nervous system lymphoma pathophysiology On the Web |
American Roentgen Ray Society Images of Primary central nervous system lymphoma pathophysiology |
FDA on Primary central nervous system lymphoma pathophysiology |
CDC on Primary central nervous system lymphoma pathophysiology |
Primary central nervous system lymphoma pathophysiology in the news |
Blogs on Primary central nervous system lymphoma pathophysiology |
Directions to Hospitals Treating Primary central nervous system lymphoma |
Risk calculators and risk factors for Primary central nervous system lymphoma pathophysiology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
Pathogenesis
- Primary central nervous system lymphoma may originate from a germinal center to an early postgerminal center in a secondary lymphoid organ, which may be capable of further maturation steps.[1]
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).[2]
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).[3]
- The lesion is mainly located at supratentorial level, usually in the periventricular regions, infiltrating the corpus callosum and the basal ganglia.[4]
- Multiple lesions are reported in 38%–55% of non-AIDS primary CNS lymphomas. Multifocal intraparenchymal lesions, without a dural involvement, are very rare. Frontal lobe is affected in 20%–43% of primary CNS lymphoma. Other sites iclude brain stem, cerebellum, leptomeninges, spinal cord, and eyes.
- 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.[3]
Gallery
-
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.[5]
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.[3]
- Malignant cells tend to accumulate around blood vessels.
- Low-grade tumors are more frequently T-cell in origin.[3]
- On microscopic histopathological analysis, primary central nervous system lymphoma is characterized by a perivascular distribution composed of large cells with prominent nucleoli.[2]
Gallery
-
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.[2]
Immunohistochemistry
Primary central nervous system lymphoma is demonstrated by positivity to tumor markers such as:[6]
References
- ↑ PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMAS IN THE NEUROLOGICAL PRACTICE. file:///C:/Users/Owner/Downloads/psyneur_2014_1_2_8.pdf. Accessed on February 19, 2016
- ↑ 2.0 2.1 2.2 Primary central nervous system lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Primary_central_nervous_system_lymphoma. Accessed on February 18, 2016
- ↑ 3.0 3.1 3.2 3.3 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
- ↑ Haldorsen, Ingfrid S; Kråkenes, Jostein; Goplen, Anne K; Dunlop, Oona; Mella, Olav; Espeland, Ansgar (2008). "AIDS-related primary central nervous system lymphoma: a Norwegian national survey 1989–2003". BMC Cancer. 8 (1): 225. doi:10.1186/1471-2407-8-225. ISSN 1471-2407.