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Liposarcomas of the mediastinum

Liposarcoma is a malignant tumor of adipocytic differentiation that rarely arises within the mediastinum. Most of the existing data available comes from scattered case reports and a few small series. The World Health Organization (WHO) recognizes four basic types of liposarcoma: well-differentiated/...

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Autores principales: Suster, David Ilan, Suster, Saul
Formato: Online Artículo Texto
Lenguaje:English
Publicado: AME Publishing Company 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8794306/
https://www.ncbi.nlm.nih.gov/pubmed/35118295
http://dx.doi.org/10.21037/med-20-42
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author Suster, David Ilan
Suster, Saul
author_facet Suster, David Ilan
Suster, Saul
author_sort Suster, David Ilan
collection PubMed
description Liposarcoma is a malignant tumor of adipocytic differentiation that rarely arises within the mediastinum. Most of the existing data available comes from scattered case reports and a few small series. The World Health Organization (WHO) recognizes four basic types of liposarcoma: well-differentiated/atypical lipomatous tumor (ALT), dedifferentiated, myxoid and pleomorphic liposarcoma (PLS). All of these liposarcoma subtypes have been recorded to occur within the mediastinum. On morphologic grounds liposarcoma can present a challenge for diagnosis as it can be difficult to distinguish from benign adipocytic neoplasms, or in the case of dedifferentiated liposarcoma (DDLS), from virtually any other type of sarcoma. Molecularly the most common subtypes of liposarcoma are characterized by specific, recurrent genetic alterations involving amplification events of MDM2 and CDK4 in well-differentiated liposarcoma (WDL) and a recurrent t(12;16)(q13;p11) in myxoid liposarcoma (MLS). MDM2 and CDK4 amplification can be assessed by immunohistochemistry, fluorescence in situ hybridization, or molecular techniques that evaluate copy number alterations and amplifications such as array based assays and next generation sequencing (NGS). In addition to WDL and MLS, a few additional rare subtypes of liposarcoma may occur in the mediastinum including PLS, myxoid WDL, thymoliposarcoma, and sclerosing high-grade liposarcoma. The present review will focus on the clinicopathologic features of the various histologic types of liposarcoma described in the mediastinum and their differential diagnosis. Data is derived from review of the largest series published in the more recent literature on these tumors.
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spelling pubmed-87943062022-02-02 Liposarcomas of the mediastinum Suster, David Ilan Suster, Saul Mediastinum Review Article Liposarcoma is a malignant tumor of adipocytic differentiation that rarely arises within the mediastinum. Most of the existing data available comes from scattered case reports and a few small series. The World Health Organization (WHO) recognizes four basic types of liposarcoma: well-differentiated/atypical lipomatous tumor (ALT), dedifferentiated, myxoid and pleomorphic liposarcoma (PLS). All of these liposarcoma subtypes have been recorded to occur within the mediastinum. On morphologic grounds liposarcoma can present a challenge for diagnosis as it can be difficult to distinguish from benign adipocytic neoplasms, or in the case of dedifferentiated liposarcoma (DDLS), from virtually any other type of sarcoma. Molecularly the most common subtypes of liposarcoma are characterized by specific, recurrent genetic alterations involving amplification events of MDM2 and CDK4 in well-differentiated liposarcoma (WDL) and a recurrent t(12;16)(q13;p11) in myxoid liposarcoma (MLS). MDM2 and CDK4 amplification can be assessed by immunohistochemistry, fluorescence in situ hybridization, or molecular techniques that evaluate copy number alterations and amplifications such as array based assays and next generation sequencing (NGS). In addition to WDL and MLS, a few additional rare subtypes of liposarcoma may occur in the mediastinum including PLS, myxoid WDL, thymoliposarcoma, and sclerosing high-grade liposarcoma. The present review will focus on the clinicopathologic features of the various histologic types of liposarcoma described in the mediastinum and their differential diagnosis. Data is derived from review of the largest series published in the more recent literature on these tumors. AME Publishing Company 2020-09-30 /pmc/articles/PMC8794306/ /pubmed/35118295 http://dx.doi.org/10.21037/med-20-42 Text en 2020 Mediastinum. All rights reserved. https://creativecommons.org/licenses/by-nc-nd/4.0/Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.
spellingShingle Review Article
Suster, David Ilan
Suster, Saul
Liposarcomas of the mediastinum
title Liposarcomas of the mediastinum
title_full Liposarcomas of the mediastinum
title_fullStr Liposarcomas of the mediastinum
title_full_unstemmed Liposarcomas of the mediastinum
title_short Liposarcomas of the mediastinum
title_sort liposarcomas of the mediastinum
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8794306/
https://www.ncbi.nlm.nih.gov/pubmed/35118295
http://dx.doi.org/10.21037/med-20-42
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