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Advances in Pathobiology of Primary Central Nervous System Lymphoma

OBJECTIVE: Primary central nervous system lymphoma (PCNSL) is a specific type of non-Hodgkin lymphoma with poor prognosis. The rare incidence of this disease and difficulty to obtain sufficient tissue material impede deep research into PCNSL. However, application of modern molecular techniques makes...

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Autores principales: Yang, Xue-Liang, Liu, Yuan-Bo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5555133/
https://www.ncbi.nlm.nih.gov/pubmed/28776551
http://dx.doi.org/10.4103/0366-6999.211879
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author Yang, Xue-Liang
Liu, Yuan-Bo
author_facet Yang, Xue-Liang
Liu, Yuan-Bo
author_sort Yang, Xue-Liang
collection PubMed
description OBJECTIVE: Primary central nervous system lymphoma (PCNSL) is a specific type of non-Hodgkin lymphoma with poor prognosis. The rare incidence of this disease and difficulty to obtain sufficient tissue material impede deep research into PCNSL. However, application of modern molecular techniques makes it possible to find biological characteristics exclusive to PCNSL. Therefore, we systematically reviewed the latest research progress on biological characteristics and pathogenesis of PCNSL. DATA SOURCES: The data analyzed in this review were from the articles listed in PubMed database. STUDY SELECTION: Articles focusing on the biology of PCNSL at the cytogenetic or molecular level were reviewed, including clinical, basic research, and review articles. RESULTS: With respect to histopathology, perivascular growth pattern and reactive perivascular T-cell infiltration are regarded as typical histopathological manifestations of tumor cells in PCNSL. Moreover, tumor cells of PCNSL predominantly express an activated B-cell-like phenotype, including CD10(−) BCL-6(+) MUM1(+), CD10(−) BCL-6(−) MUM1(+), and CD10(−) BCL-6(−) MUM1(−). On the molecular level, some molecular and genetic alterations may contribute to malignant transformation, including mutations of proto-oncogenes and tumor suppressor genes, gains and losses of genetic material, as well as aberrant activation of some important signaling pathways, such as nuclear factor-κB and JAK/STAT pathway. CONCLUSIONS: The integrated molecular mechanisms involved in pathogenesis of PCNSL are not well understood. The important biomarkers indicating prognosis are not identified. Multicenter studies should be carried out to elucidate pathogenesis of PCNSL to find novel and effective therapeutic strategies.
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spelling pubmed-55551332017-08-31 Advances in Pathobiology of Primary Central Nervous System Lymphoma Yang, Xue-Liang Liu, Yuan-Bo Chin Med J (Engl) Review Article OBJECTIVE: Primary central nervous system lymphoma (PCNSL) is a specific type of non-Hodgkin lymphoma with poor prognosis. The rare incidence of this disease and difficulty to obtain sufficient tissue material impede deep research into PCNSL. However, application of modern molecular techniques makes it possible to find biological characteristics exclusive to PCNSL. Therefore, we systematically reviewed the latest research progress on biological characteristics and pathogenesis of PCNSL. DATA SOURCES: The data analyzed in this review were from the articles listed in PubMed database. STUDY SELECTION: Articles focusing on the biology of PCNSL at the cytogenetic or molecular level were reviewed, including clinical, basic research, and review articles. RESULTS: With respect to histopathology, perivascular growth pattern and reactive perivascular T-cell infiltration are regarded as typical histopathological manifestations of tumor cells in PCNSL. Moreover, tumor cells of PCNSL predominantly express an activated B-cell-like phenotype, including CD10(−) BCL-6(+) MUM1(+), CD10(−) BCL-6(−) MUM1(+), and CD10(−) BCL-6(−) MUM1(−). On the molecular level, some molecular and genetic alterations may contribute to malignant transformation, including mutations of proto-oncogenes and tumor suppressor genes, gains and losses of genetic material, as well as aberrant activation of some important signaling pathways, such as nuclear factor-κB and JAK/STAT pathway. CONCLUSIONS: The integrated molecular mechanisms involved in pathogenesis of PCNSL are not well understood. The important biomarkers indicating prognosis are not identified. Multicenter studies should be carried out to elucidate pathogenesis of PCNSL to find novel and effective therapeutic strategies. Medknow Publications & Media Pvt Ltd 2017-08-20 /pmc/articles/PMC5555133/ /pubmed/28776551 http://dx.doi.org/10.4103/0366-6999.211879 Text en Copyright: © 2017 Chinese Medical Journal http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle Review Article
Yang, Xue-Liang
Liu, Yuan-Bo
Advances in Pathobiology of Primary Central Nervous System Lymphoma
title Advances in Pathobiology of Primary Central Nervous System Lymphoma
title_full Advances in Pathobiology of Primary Central Nervous System Lymphoma
title_fullStr Advances in Pathobiology of Primary Central Nervous System Lymphoma
title_full_unstemmed Advances in Pathobiology of Primary Central Nervous System Lymphoma
title_short Advances in Pathobiology of Primary Central Nervous System Lymphoma
title_sort advances in pathobiology of primary central nervous system lymphoma
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5555133/
https://www.ncbi.nlm.nih.gov/pubmed/28776551
http://dx.doi.org/10.4103/0366-6999.211879
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