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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/...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
AME Publishing Company
2020
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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. |
format | Online Article Text |
id | pubmed-8794306 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | AME Publishing Company |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT susterdavidilan liposarcomasofthemediastinum AT sustersaul liposarcomasofthemediastinum |