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Case report: Kidney perivascular epithelioid cell tumor treated with anti-VEGFR tyrosine kinase inhibitor and MTOR inhibitor
Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors arising from perivascular epithelial cells. There was no standard treatment for unresectable PEComa before 2021. For a low incidence and a rarely curable disease, development of new therapy is essential. A 45-year-old female...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9709202/ https://www.ncbi.nlm.nih.gov/pubmed/36465390 http://dx.doi.org/10.3389/fonc.2022.966818 |
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author | Zhang, Ninggang Ren, Yaqiong Zan, Likun Zhang, Xuting Zhao, Jian Wen, Lu Wang, Yusheng |
author_facet | Zhang, Ninggang Ren, Yaqiong Zan, Likun Zhang, Xuting Zhao, Jian Wen, Lu Wang, Yusheng |
author_sort | Zhang, Ninggang |
collection | PubMed |
description | Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors arising from perivascular epithelial cells. There was no standard treatment for unresectable PEComa before 2021. For a low incidence and a rarely curable disease, development of new therapy is essential. A 45-year-old female was diagnosed with malignant renal PEComa (likely with TFE3 rearrangement) that underwent rapid progression after 10 months of surgery. The patient then received the tyrosine kinase inhibitor (TKI) Apatinib, and the tumor remained stable for 15 months before another progression. The patient then received the MTOR inhibitor everolimus that alleviated her symptoms but the tumor went into remission again after another 15 months. This result suggests that antagonizing the vascular endothelial growth factor receptor (VEGFR) pathway be a useful strategy for malignant PEComas, along with the MTOR pathway inhibition that had recently been approved for the rare tumor. |
format | Online Article Text |
id | pubmed-9709202 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-97092022022-12-01 Case report: Kidney perivascular epithelioid cell tumor treated with anti-VEGFR tyrosine kinase inhibitor and MTOR inhibitor Zhang, Ninggang Ren, Yaqiong Zan, Likun Zhang, Xuting Zhao, Jian Wen, Lu Wang, Yusheng Front Oncol Oncology Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors arising from perivascular epithelial cells. There was no standard treatment for unresectable PEComa before 2021. For a low incidence and a rarely curable disease, development of new therapy is essential. A 45-year-old female was diagnosed with malignant renal PEComa (likely with TFE3 rearrangement) that underwent rapid progression after 10 months of surgery. The patient then received the tyrosine kinase inhibitor (TKI) Apatinib, and the tumor remained stable for 15 months before another progression. The patient then received the MTOR inhibitor everolimus that alleviated her symptoms but the tumor went into remission again after another 15 months. This result suggests that antagonizing the vascular endothelial growth factor receptor (VEGFR) pathway be a useful strategy for malignant PEComas, along with the MTOR pathway inhibition that had recently been approved for the rare tumor. Frontiers Media S.A. 2022-11-16 /pmc/articles/PMC9709202/ /pubmed/36465390 http://dx.doi.org/10.3389/fonc.2022.966818 Text en Copyright © 2022 Zhang, Ren, Zan, Zhang, Zhao, Wen and Wang https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Oncology Zhang, Ninggang Ren, Yaqiong Zan, Likun Zhang, Xuting Zhao, Jian Wen, Lu Wang, Yusheng Case report: Kidney perivascular epithelioid cell tumor treated with anti-VEGFR tyrosine kinase inhibitor and MTOR inhibitor |
title | Case report: Kidney perivascular epithelioid cell tumor treated with anti-VEGFR tyrosine kinase inhibitor and MTOR inhibitor |
title_full | Case report: Kidney perivascular epithelioid cell tumor treated with anti-VEGFR tyrosine kinase inhibitor and MTOR inhibitor |
title_fullStr | Case report: Kidney perivascular epithelioid cell tumor treated with anti-VEGFR tyrosine kinase inhibitor and MTOR inhibitor |
title_full_unstemmed | Case report: Kidney perivascular epithelioid cell tumor treated with anti-VEGFR tyrosine kinase inhibitor and MTOR inhibitor |
title_short | Case report: Kidney perivascular epithelioid cell tumor treated with anti-VEGFR tyrosine kinase inhibitor and MTOR inhibitor |
title_sort | case report: kidney perivascular epithelioid cell tumor treated with anti-vegfr tyrosine kinase inhibitor and mtor inhibitor |
topic | Oncology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9709202/ https://www.ncbi.nlm.nih.gov/pubmed/36465390 http://dx.doi.org/10.3389/fonc.2022.966818 |
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