Primary central nervous system lymphoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Primary CNS lymphoma is a primary intracranial tumor usually present in those with severe immunosuppression --- commonly in those with AIDS --- and represents around 20% of all cases of lymphomas in HIV infection (other types being Burkitt's lymphoma and immunoblastic lymphoma).
Classification
Primary central nervous system lymphoma may be classified according to the site of involvement of the central nervous system into 3 groups: parenchymal, intravascular lymphoma, and primary leptomeningeal lymphoma.[1]
Pathophysiology
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.[2] EBV is almost always associated with primary CNS lymphoma in immunodeficient individuals, and latent EBV infection of B-cells leads to it's immortalization and to CNS tropism. In immunocompetent individuals, the B-cells infected with EBV are held in check by T-cells, and with the severity of immunosuppression, T cells gradually fall, leading to the B-cell proliferation and dissemination.[3] Genes involved in the pathogenesis of primary central nervous system lymphoma include IG, BCL6, CD95, PAX5, and PIM1.[4] 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).[5] Primary central nervous system lymphoma presents as a solitary or multiple, well circumscribed, greater than 2 cm in diameter, 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).[6][3] On gross pathology, the lesions could be brownish, gray-tan, or yellow, firm, homogenous, centrally necrotic with areas of hemorrhage.[3] On microscopic histopathological analysis, primary central nervous system lymphoma is characterized by accumulation of large atypical mononuclear cells around small calibre vascular channels with invasion of the walls of some vessels. The atypical cells have large round, oval and angulated hyperchromatic and vesicular nuclei, and a narrow rim of pale cytoplasm. An occasional mitotic figure is noted.[5][7] Primary central nervous system lymphoma is demonstrated by positivity to tumor markers, including B lymphocyte markers, such as CD19, CD20, CD79a, Ki-67, GFAP, BCL-2, and BCL-6.[3][7][8][9]
Causes
There are no known direct causes for primary central nervous system lymphoma. To view a comprehensive list of risk factors that increase the risk of primary nervous system lymphoma, click here.[10][11][12]
Differentiating Primary Central Nervous System Lymphoma from other Diseases
Primary central nervous system lymphoma must be differentiated from secondary CNS lymphoma, cerebral toxoplasmosis, glioblastoma multiforme, cerebral abscess, and tuberculoma.[13][14]
Risk Factors
References
- ↑ CNS lymphoma. Dr Henry Knipe and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/cns-lymphoma-1. Accessed on February 17, 2016
- ↑ 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
- ↑ 3.0 3.1 3.2 3.3 Bhagavathi S, Wilson JD (2008). "Primary central nervous system lymphoma". Arch Pathol Lab Med. 132 (11): 1830–4. doi:10.1043/1543-2165-132.11.1830. PMID 18976024.
- ↑ Ferreri, A. J. M. (2011). "How I treat primary CNS lymphoma". Blood. 118 (3): 510–522. doi:10.1182/blood-2011-03-321349. ISSN 0006-4971.
- ↑ 5.0 5.1 Primary central nervous system lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Primary_central_nervous_system_lymphoma. Accessed on February 18, 2016
- ↑ 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
- ↑ 7.0 7.1 CNS lymphoma (primary). A.Prof Frank Gaillard. Radioapaedia 2016. http://radiopaedia.org/cases/cns-lymphoma-primary. Accessed on February 23, 2016
- ↑ 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.
- ↑ Yamanaka, Ryuya (2013). "Primary Central Nervous System Lymphoma − Recent Advance on Clinical Research". doi:10.5772/52757.
- ↑ Risks of primary central nervous lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 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.
- ↑ Epidemiology of primary CNS 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
- ↑ Differential diagnosis of primary central nervous system lymphoma. Dr Amir Rezaee and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/primary-cns-lymphoma. Accessed on February 17, 2016
- ↑ Cerebral ring enhancing lesions. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radioapaedia 2016. http://radiopaedia.org/articles/cerebral-ring-enhancing-lesions. Accessed on February 17, 2016