Cargando…
Clinical features of 18 perivascular epithelioid cell tumor cases
To investigate the biological behavior and clinical characteristics of perivascular epithelioid cell tumor (PEComa). Eighteen PEComa patients admitted to Zhongshan Hospital and the Central Hospital of Xuhui District in China from January 2006 to October 2018 were included. All patients were diagnose...
Autores principales: | , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Lippincott Williams & Wilkins
2020
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7447485/ https://www.ncbi.nlm.nih.gov/pubmed/32846779 http://dx.doi.org/10.1097/MD.0000000000021659 |
_version_ | 1783574311850737664 |
---|---|
author | Jia, Rongfei Jiang, Ling Zhou, Yuhong Wang, Yan Guo, Xi Ji, Yuan Ni, Xiang Yang, Xiaoyan |
author_facet | Jia, Rongfei Jiang, Ling Zhou, Yuhong Wang, Yan Guo, Xi Ji, Yuan Ni, Xiang Yang, Xiaoyan |
author_sort | Jia, Rongfei |
collection | PubMed |
description | To investigate the biological behavior and clinical characteristics of perivascular epithelioid cell tumor (PEComa). Eighteen PEComa patients admitted to Zhongshan Hospital and the Central Hospital of Xuhui District in China from January 2006 to October 2018 were included. All patients were diagnosed based on pathological findings and treated with surgical resection or medication. Among the 18 patients, 1 underwent lymph node biopsy for multiple enlarged lymph nodes and 17 underwent mass resection. The median disease-free survival was 22 months after the first resection and over 12 months following a second resection. Treatment with mechanistic target of rapamycin (mTOR) inhibitors was effective for patients with unresectable or metastatic lesions. The median progression-free survival was approximately 13 months. Surgery is the predominant treatment approach for PEComa and patients can benefit from multiple operations. mTOR inhibitors are considered for patients with multiple lesions or intolerance to surgery. Anti-angiogenetic drugs can be selected when mTOR inhibitors fail to control the illness. |
format | Online Article Text |
id | pubmed-7447485 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Lippincott Williams & Wilkins |
record_format | MEDLINE/PubMed |
spelling | pubmed-74474852020-09-04 Clinical features of 18 perivascular epithelioid cell tumor cases Jia, Rongfei Jiang, Ling Zhou, Yuhong Wang, Yan Guo, Xi Ji, Yuan Ni, Xiang Yang, Xiaoyan Medicine (Baltimore) 5700 To investigate the biological behavior and clinical characteristics of perivascular epithelioid cell tumor (PEComa). Eighteen PEComa patients admitted to Zhongshan Hospital and the Central Hospital of Xuhui District in China from January 2006 to October 2018 were included. All patients were diagnosed based on pathological findings and treated with surgical resection or medication. Among the 18 patients, 1 underwent lymph node biopsy for multiple enlarged lymph nodes and 17 underwent mass resection. The median disease-free survival was 22 months after the first resection and over 12 months following a second resection. Treatment with mechanistic target of rapamycin (mTOR) inhibitors was effective for patients with unresectable or metastatic lesions. The median progression-free survival was approximately 13 months. Surgery is the predominant treatment approach for PEComa and patients can benefit from multiple operations. mTOR inhibitors are considered for patients with multiple lesions or intolerance to surgery. Anti-angiogenetic drugs can be selected when mTOR inhibitors fail to control the illness. Lippincott Williams & Wilkins 2020-08-21 /pmc/articles/PMC7447485/ /pubmed/32846779 http://dx.doi.org/10.1097/MD.0000000000021659 Text en Copyright © 2020 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by-nc/4.0 This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc/4.0 |
spellingShingle | 5700 Jia, Rongfei Jiang, Ling Zhou, Yuhong Wang, Yan Guo, Xi Ji, Yuan Ni, Xiang Yang, Xiaoyan Clinical features of 18 perivascular epithelioid cell tumor cases |
title | Clinical features of 18 perivascular epithelioid cell tumor cases |
title_full | Clinical features of 18 perivascular epithelioid cell tumor cases |
title_fullStr | Clinical features of 18 perivascular epithelioid cell tumor cases |
title_full_unstemmed | Clinical features of 18 perivascular epithelioid cell tumor cases |
title_short | Clinical features of 18 perivascular epithelioid cell tumor cases |
title_sort | clinical features of 18 perivascular epithelioid cell tumor cases |
topic | 5700 |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7447485/ https://www.ncbi.nlm.nih.gov/pubmed/32846779 http://dx.doi.org/10.1097/MD.0000000000021659 |
work_keys_str_mv | AT jiarongfei clinicalfeaturesof18perivascularepithelioidcelltumorcases AT jiangling clinicalfeaturesof18perivascularepithelioidcelltumorcases AT zhouyuhong clinicalfeaturesof18perivascularepithelioidcelltumorcases AT wangyan clinicalfeaturesof18perivascularepithelioidcelltumorcases AT guoxi clinicalfeaturesof18perivascularepithelioidcelltumorcases AT jiyuan clinicalfeaturesof18perivascularepithelioidcelltumorcases AT nixiang clinicalfeaturesof18perivascularepithelioidcelltumorcases AT yangxiaoyan clinicalfeaturesof18perivascularepithelioidcelltumorcases |