Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Zhang, Ninggang, Ren, Yaqiong, Zan, Likun, Zhang, Xuting, Zhao, Jian, Wen, Lu, Wang, Yusheng
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
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
_version_ 1784841095977369600
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
work_keys_str_mv AT zhangninggang casereportkidneyperivascularepithelioidcelltumortreatedwithantivegfrtyrosinekinaseinhibitorandmtorinhibitor
AT renyaqiong casereportkidneyperivascularepithelioidcelltumortreatedwithantivegfrtyrosinekinaseinhibitorandmtorinhibitor
AT zanlikun casereportkidneyperivascularepithelioidcelltumortreatedwithantivegfrtyrosinekinaseinhibitorandmtorinhibitor
AT zhangxuting casereportkidneyperivascularepithelioidcelltumortreatedwithantivegfrtyrosinekinaseinhibitorandmtorinhibitor
AT zhaojian casereportkidneyperivascularepithelioidcelltumortreatedwithantivegfrtyrosinekinaseinhibitorandmtorinhibitor
AT wenlu casereportkidneyperivascularepithelioidcelltumortreatedwithantivegfrtyrosinekinaseinhibitorandmtorinhibitor
AT wangyusheng casereportkidneyperivascularepithelioidcelltumortreatedwithantivegfrtyrosinekinaseinhibitorandmtorinhibitor