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Epithelioid hemangioendothelioma of the retroperitoneal giant type treated with Toripalimab: A case report
Epithelioid hemangioendotheliomas (EHEs), low-grade malignant tumors of vascular endothelial cell origin, are characterized by vascular endothelial proliferation. In 2002, the World Health Organization classified EHEs as locally aggressive tumors with the potential to metastasize. Currently, the dia...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2023
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10063782/ https://www.ncbi.nlm.nih.gov/pubmed/37006308 http://dx.doi.org/10.3389/fimmu.2023.1116944 |
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author | Bu, Yuqing Peng, Lili Liu, Miaomiao He, Liya Wang, Fayan Li, Bingjie Niu, Xueliang Zhang, Hongzhen |
author_facet | Bu, Yuqing Peng, Lili Liu, Miaomiao He, Liya Wang, Fayan Li, Bingjie Niu, Xueliang Zhang, Hongzhen |
author_sort | Bu, Yuqing |
collection | PubMed |
description | Epithelioid hemangioendotheliomas (EHEs), low-grade malignant tumors of vascular endothelial cell origin, are characterized by vascular endothelial proliferation. In 2002, the World Health Organization classified EHEs as locally aggressive tumors with the potential to metastasize. Currently, the diagnosis of EHE is based on pathology, histological and immunohistochemical examinations. There are no standard treatment guidelines. We here report a 69-year-old man who presented with left-sided chest and abdominal pain for more than 2 months. Enhanced computed tomography of the thorax and abdomen in another hospital suggested a mass in the left adrenal region that was considered malignant. Positron emission tomography- computed tomography in our hospital suggested a large multi-loculated, hypermetabolic, cystic mass in the left adrenal region that was considered malignant. Accordingly, a puncture biopsy of the mass was performed and the diagnosis of EHE confirmed by pathological examination, including immunohistochemical staining. This patient was treated with the programmed death 1 (PD-1) immune checkpoint inhibitor toripalimab with long-term success. The best response was stable disease (SD) with a progression-free survival (PFS) of more than 13 months. The patient is still alive now. Because the sample size of previous studies was small, further studies are needed to determine the safety and efficacy of toripalimab in the treatment of EHE. |
format | Online Article Text |
id | pubmed-10063782 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-100637822023-04-01 Epithelioid hemangioendothelioma of the retroperitoneal giant type treated with Toripalimab: A case report Bu, Yuqing Peng, Lili Liu, Miaomiao He, Liya Wang, Fayan Li, Bingjie Niu, Xueliang Zhang, Hongzhen Front Immunol Immunology Epithelioid hemangioendotheliomas (EHEs), low-grade malignant tumors of vascular endothelial cell origin, are characterized by vascular endothelial proliferation. In 2002, the World Health Organization classified EHEs as locally aggressive tumors with the potential to metastasize. Currently, the diagnosis of EHE is based on pathology, histological and immunohistochemical examinations. There are no standard treatment guidelines. We here report a 69-year-old man who presented with left-sided chest and abdominal pain for more than 2 months. Enhanced computed tomography of the thorax and abdomen in another hospital suggested a mass in the left adrenal region that was considered malignant. Positron emission tomography- computed tomography in our hospital suggested a large multi-loculated, hypermetabolic, cystic mass in the left adrenal region that was considered malignant. Accordingly, a puncture biopsy of the mass was performed and the diagnosis of EHE confirmed by pathological examination, including immunohistochemical staining. This patient was treated with the programmed death 1 (PD-1) immune checkpoint inhibitor toripalimab with long-term success. The best response was stable disease (SD) with a progression-free survival (PFS) of more than 13 months. The patient is still alive now. Because the sample size of previous studies was small, further studies are needed to determine the safety and efficacy of toripalimab in the treatment of EHE. Frontiers Media S.A. 2023-03-17 /pmc/articles/PMC10063782/ /pubmed/37006308 http://dx.doi.org/10.3389/fimmu.2023.1116944 Text en Copyright © 2023 Bu, Peng, Liu, He, Wang, Li, Niu and Zhang 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 | Immunology Bu, Yuqing Peng, Lili Liu, Miaomiao He, Liya Wang, Fayan Li, Bingjie Niu, Xueliang Zhang, Hongzhen Epithelioid hemangioendothelioma of the retroperitoneal giant type treated with Toripalimab: A case report |
title | Epithelioid hemangioendothelioma of the retroperitoneal giant type treated with Toripalimab: A case report |
title_full | Epithelioid hemangioendothelioma of the retroperitoneal giant type treated with Toripalimab: A case report |
title_fullStr | Epithelioid hemangioendothelioma of the retroperitoneal giant type treated with Toripalimab: A case report |
title_full_unstemmed | Epithelioid hemangioendothelioma of the retroperitoneal giant type treated with Toripalimab: A case report |
title_short | Epithelioid hemangioendothelioma of the retroperitoneal giant type treated with Toripalimab: A case report |
title_sort | epithelioid hemangioendothelioma of the retroperitoneal giant type treated with toripalimab: a case report |
topic | Immunology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10063782/ https://www.ncbi.nlm.nih.gov/pubmed/37006308 http://dx.doi.org/10.3389/fimmu.2023.1116944 |
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