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Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases

Dedifferentiated liposarcoma (DDL) is a histologically pleomorphic sarcoma, traditionally defined as well-differentiated liposarcoma with abrupt transition to high grade, non-lipogenic sarcoma. It can occur as part of recurrent well-differentiated liposarcoma, or may arise de novo. DDL most frequent...

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Autores principales: Ben Salha, Imen, Zaidi, Shane, Noujaim, Jonathan, Miah, Aisha B., Fisher, Cyril, Jones, Robin L., Thway, Khin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: PAGEPress Publications, Pavia, Italy 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5064295/
https://www.ncbi.nlm.nih.gov/pubmed/27746879
http://dx.doi.org/10.4081/rt.2016.6282
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author Ben Salha, Imen
Zaidi, Shane
Noujaim, Jonathan
Miah, Aisha B.
Fisher, Cyril
Jones, Robin L.
Thway, Khin
author_facet Ben Salha, Imen
Zaidi, Shane
Noujaim, Jonathan
Miah, Aisha B.
Fisher, Cyril
Jones, Robin L.
Thway, Khin
author_sort Ben Salha, Imen
collection PubMed
description Dedifferentiated liposarcoma (DDL) is a histologically pleomorphic sarcoma, traditionally defined as well-differentiated liposarcoma with abrupt transition to high grade, non-lipogenic sarcoma. It can occur as part of recurrent well-differentiated liposarcoma, or may arise de novo. DDL most frequently occurs within the retroperitoneum, and while it is prone to local recurrence, it usually has a lower rate of metastasis than other pleomorphic sarcomas. We describe a case of retroperitoneal dedifferentiated liposarcoma in a 63-year-old male, who showed MDM2 amplification with fluorescence in situ hybridization, which displayed unusually aggressive behavior, with brain, lung and subcutaneous soft tissue metastases. As previous reports of metastatic liposarcoma have largely grouped DDL in with other (genetically and clinically distinct) liposarcoma subtypes, we highlight and discuss the rare occurrence of brain metastasis in MDM2-amplified retroperitoneal liposarcoma.
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spelling pubmed-50642952016-10-14 Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases Ben Salha, Imen Zaidi, Shane Noujaim, Jonathan Miah, Aisha B. Fisher, Cyril Jones, Robin L. Thway, Khin Rare Tumors Case Report Dedifferentiated liposarcoma (DDL) is a histologically pleomorphic sarcoma, traditionally defined as well-differentiated liposarcoma with abrupt transition to high grade, non-lipogenic sarcoma. It can occur as part of recurrent well-differentiated liposarcoma, or may arise de novo. DDL most frequently occurs within the retroperitoneum, and while it is prone to local recurrence, it usually has a lower rate of metastasis than other pleomorphic sarcomas. We describe a case of retroperitoneal dedifferentiated liposarcoma in a 63-year-old male, who showed MDM2 amplification with fluorescence in situ hybridization, which displayed unusually aggressive behavior, with brain, lung and subcutaneous soft tissue metastases. As previous reports of metastatic liposarcoma have largely grouped DDL in with other (genetically and clinically distinct) liposarcoma subtypes, we highlight and discuss the rare occurrence of brain metastasis in MDM2-amplified retroperitoneal liposarcoma. PAGEPress Publications, Pavia, Italy 2016-10-05 /pmc/articles/PMC5064295/ /pubmed/27746879 http://dx.doi.org/10.4081/rt.2016.6282 Text en ©Copyright I.B. Salha et al. http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Ben Salha, Imen
Zaidi, Shane
Noujaim, Jonathan
Miah, Aisha B.
Fisher, Cyril
Jones, Robin L.
Thway, Khin
Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases
title Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases
title_full Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases
title_fullStr Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases
title_full_unstemmed Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases
title_short Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases
title_sort rare aggressive behavior of mdm2-amplified retroperitoneal dedifferentiated liposarcoma, with brain, lung and subcutaneous metastases
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5064295/
https://www.ncbi.nlm.nih.gov/pubmed/27746879
http://dx.doi.org/10.4081/rt.2016.6282
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