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Intracranial non-myxoid angiomatoid fibrous histiocytoma with EWSR1-CREB1 transcript fusion treated with doxorubicin: A case report
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that has only been reported in the central nervous system in case reports. After surgery, patients exhibit tumor recurrence. Pathological diagnosis of AHF remains difficult, especially in sites other than skin. AFH can harbor charact...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
D.A. Spandidos
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8138849/ https://www.ncbi.nlm.nih.gov/pubmed/34055346 http://dx.doi.org/10.3892/mco.2021.2293 |
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author | Garnier, Louis Fenouil, Tanguy Pissaloux, Daniel Ameli, Roxana Ducray, François Meyronet, David Honnorat, Jerome |
author_facet | Garnier, Louis Fenouil, Tanguy Pissaloux, Daniel Ameli, Roxana Ducray, François Meyronet, David Honnorat, Jerome |
author_sort | Garnier, Louis |
collection | PubMed |
description | Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that has only been reported in the central nervous system in case reports. After surgery, patients exhibit tumor recurrence. Pathological diagnosis of AHF remains difficult, especially in sites other than skin. AFH can harbor characteristic translocations implying that the Ewing sarcoma breakpoint region 1 gene (EWSR1) fuses with the transcription factor cyclic AMP response element binding (CREB) family genes. Doxorubicin is a chemotherapy that has previously been used successfully in two metastatic soft tissue AFH cases but never in intracranial AFH. The present report describes a case of an adult with a progressive classical intracranial non-myxoid AFH with ESWR1-CREB1 transcript fusion 4 years after surgery. The patient was treated with doxorubicin as a single agent chemotherapy. This treatment resulted in a prolonged stable disease 15 months after treatment discontinuation. This is the first reported case of a treatment with doxorubicin in an adult with progressive intracranial AFH with ESWR1-CREB1 transcript fusion which was sustained after treatment discontinuation. |
format | Online Article Text |
id | pubmed-8138849 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | D.A. Spandidos |
record_format | MEDLINE/PubMed |
spelling | pubmed-81388492021-05-27 Intracranial non-myxoid angiomatoid fibrous histiocytoma with EWSR1-CREB1 transcript fusion treated with doxorubicin: A case report Garnier, Louis Fenouil, Tanguy Pissaloux, Daniel Ameli, Roxana Ducray, François Meyronet, David Honnorat, Jerome Mol Clin Oncol Articles Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that has only been reported in the central nervous system in case reports. After surgery, patients exhibit tumor recurrence. Pathological diagnosis of AHF remains difficult, especially in sites other than skin. AFH can harbor characteristic translocations implying that the Ewing sarcoma breakpoint region 1 gene (EWSR1) fuses with the transcription factor cyclic AMP response element binding (CREB) family genes. Doxorubicin is a chemotherapy that has previously been used successfully in two metastatic soft tissue AFH cases but never in intracranial AFH. The present report describes a case of an adult with a progressive classical intracranial non-myxoid AFH with ESWR1-CREB1 transcript fusion 4 years after surgery. The patient was treated with doxorubicin as a single agent chemotherapy. This treatment resulted in a prolonged stable disease 15 months after treatment discontinuation. This is the first reported case of a treatment with doxorubicin in an adult with progressive intracranial AFH with ESWR1-CREB1 transcript fusion which was sustained after treatment discontinuation. D.A. Spandidos 2021-07 2021-05-09 /pmc/articles/PMC8138849/ /pubmed/34055346 http://dx.doi.org/10.3892/mco.2021.2293 Text en Copyright: © Garnier et al. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License (https://creativecommons.org/licenses/by-nc-nd/4.0/) , which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made. |
spellingShingle | Articles Garnier, Louis Fenouil, Tanguy Pissaloux, Daniel Ameli, Roxana Ducray, François Meyronet, David Honnorat, Jerome Intracranial non-myxoid angiomatoid fibrous histiocytoma with EWSR1-CREB1 transcript fusion treated with doxorubicin: A case report |
title | Intracranial non-myxoid angiomatoid fibrous histiocytoma with EWSR1-CREB1 transcript fusion treated with doxorubicin: A case report |
title_full | Intracranial non-myxoid angiomatoid fibrous histiocytoma with EWSR1-CREB1 transcript fusion treated with doxorubicin: A case report |
title_fullStr | Intracranial non-myxoid angiomatoid fibrous histiocytoma with EWSR1-CREB1 transcript fusion treated with doxorubicin: A case report |
title_full_unstemmed | Intracranial non-myxoid angiomatoid fibrous histiocytoma with EWSR1-CREB1 transcript fusion treated with doxorubicin: A case report |
title_short | Intracranial non-myxoid angiomatoid fibrous histiocytoma with EWSR1-CREB1 transcript fusion treated with doxorubicin: A case report |
title_sort | intracranial non-myxoid angiomatoid fibrous histiocytoma with ewsr1-creb1 transcript fusion treated with doxorubicin: a case report |
topic | Articles |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8138849/ https://www.ncbi.nlm.nih.gov/pubmed/34055346 http://dx.doi.org/10.3892/mco.2021.2293 |
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