Testicular Diffuse Large B-Cell Lymphoma—Clinical, Molecular, and Immunological Features

Primary testicular lymphoma is a rare lymphoma entity, yet it is the most common testicular malignancy among elderly men. The majority of the cases represent non-germinal center B-cell-like (non-GCB) diffuse large B-cell lymphoma (DLBCL) with aggressive clinical behavior and a relatively high relaps...

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Main Authors: Marjukka Pollari, Suvi-Katri Leivonen, Sirpa Leppä
Format: Article
Language:English
Published: MDPI AG 2021-08-01
Series:Cancers
Subjects:
Online Access:https://www.mdpi.com/2072-6694/13/16/4049
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author Marjukka Pollari
Suvi-Katri Leivonen
Sirpa Leppä
author_facet Marjukka Pollari
Suvi-Katri Leivonen
Sirpa Leppä
author_sort Marjukka Pollari
collection DOAJ
description Primary testicular lymphoma is a rare lymphoma entity, yet it is the most common testicular malignancy among elderly men. The majority of the cases represent non-germinal center B-cell-like (non-GCB) diffuse large B-cell lymphoma (DLBCL) with aggressive clinical behavior and a relatively high relapse rate. Due to the rareness of the disease, no randomized clinical trials have been conducted and the currently recognized standard of care is based on retrospective analyses and few phase II trials. During recent years, the tumor microenvironment (TME) and tumor-related immunity have been the focus of many tumor biology studies, and the emergence of targeted therapies and checkpoint inhibitors has significantly modulated the field of cancer therapies. Testicular DLBCL (T-DLBCL) is presented in an immune-privileged site of the testis, and the roles of NF-κB pathway signaling, 9p24.1 aberrations, and tumor-infiltrating immune cells, especially immune checkpoint expressing lymphocytes and macrophages, seem to be unique compared to other lymphoma entities. Preliminary data on the use of immune checkpoint inhibitors in the treatment of T-DLBCL are promising and more studies are ongoing.
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spelling doaj.art-be2d7a70c672496c97bd0d2beea726012023-11-22T07:03:01ZengMDPI AGCancers2072-66942021-08-011316404910.3390/cancers13164049Testicular Diffuse Large B-Cell Lymphoma—Clinical, Molecular, and Immunological FeaturesMarjukka Pollari0Suvi-Katri Leivonen1Sirpa Leppä2Research Program Unit, Faculty of Medicine, University of Helsinki, 00014 Helsinki, FinlandResearch Program Unit, Faculty of Medicine, University of Helsinki, 00014 Helsinki, FinlandResearch Program Unit, Faculty of Medicine, University of Helsinki, 00014 Helsinki, FinlandPrimary testicular lymphoma is a rare lymphoma entity, yet it is the most common testicular malignancy among elderly men. The majority of the cases represent non-germinal center B-cell-like (non-GCB) diffuse large B-cell lymphoma (DLBCL) with aggressive clinical behavior and a relatively high relapse rate. Due to the rareness of the disease, no randomized clinical trials have been conducted and the currently recognized standard of care is based on retrospective analyses and few phase II trials. During recent years, the tumor microenvironment (TME) and tumor-related immunity have been the focus of many tumor biology studies, and the emergence of targeted therapies and checkpoint inhibitors has significantly modulated the field of cancer therapies. Testicular DLBCL (T-DLBCL) is presented in an immune-privileged site of the testis, and the roles of NF-κB pathway signaling, 9p24.1 aberrations, and tumor-infiltrating immune cells, especially immune checkpoint expressing lymphocytes and macrophages, seem to be unique compared to other lymphoma entities. Preliminary data on the use of immune checkpoint inhibitors in the treatment of T-DLBCL are promising and more studies are ongoing.https://www.mdpi.com/2072-6694/13/16/4049testicular diffuse large B-cell lymphomalymphoma immunologytumor micro-environment
spellingShingle Marjukka Pollari
Suvi-Katri Leivonen
Sirpa Leppä
Testicular Diffuse Large B-Cell Lymphoma—Clinical, Molecular, and Immunological Features
Cancers
testicular diffuse large B-cell lymphoma
lymphoma immunology
tumor micro-environment
title Testicular Diffuse Large B-Cell Lymphoma—Clinical, Molecular, and Immunological Features
title_full Testicular Diffuse Large B-Cell Lymphoma—Clinical, Molecular, and Immunological Features
title_fullStr Testicular Diffuse Large B-Cell Lymphoma—Clinical, Molecular, and Immunological Features
title_full_unstemmed Testicular Diffuse Large B-Cell Lymphoma—Clinical, Molecular, and Immunological Features
title_short Testicular Diffuse Large B-Cell Lymphoma—Clinical, Molecular, and Immunological Features
title_sort testicular diffuse large b cell lymphoma clinical molecular and immunological features
topic testicular diffuse large B-cell lymphoma
lymphoma immunology
tumor micro-environment
url https://www.mdpi.com/2072-6694/13/16/4049
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AT suvikatrileivonen testiculardiffuselargebcelllymphomaclinicalmolecularandimmunologicalfeatures
AT sirpaleppa testiculardiffuselargebcelllymphomaclinicalmolecularandimmunologicalfeatures