Copyright
©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
Massive simultaneous hepatic and renal perivascular epithelioid cell tumor benefitted from surgery and everolimus treatment: A case report
Han-Teng Yang, Fu-Rong Wang, Na He, Yuan-Hua She, Yong-Yue Du, Wen-Gui Shi, Jing Yang, Gang Chen, Shu-Ze Zhang, Feng Cui, Bo Long, Ze-Yuan Yu, Jun-Min Zhu, Geng-Yuan Zhang
Han-Teng Yang, Yuan-Hua She, Yong-Yue Du, Gang Chen, Shu-Ze Zhang, Feng Cui, Bo Long, Ze-Yuan Yu, Jun-Min Zhu, Geng-Yuan Zhang, Department of General Surgery, Lanzhou University Second Hospital, Lanzhou 730030, Gansu Province, China
Fu-Rong Wang, Department of Pathology, Lanzhou University Second Hospital, Lanzhou 730030, Gansu Province, China
Na He, Oncology Department Ward, Lanzhou University Second Hospital, Lanzhou 730030, Gansu Province, China
Wen-Gui Shi, Jing Yang, Cuiying Biomedical Research Center, Lanzhou University Second Hospital, Lanzhou 730030, Gansu Province, China
Co-first authors: Han-Teng Yang and Fu-Rong Wang.
Co-corresponding authors: Jun-Min Zhu and Geng-Yuan Zhang.
Author contributions: Yang HT, Zhang GY, Wang FR, He N and Zhu JM conceptualised and wrote the original draft; She YH, Du YY, Shi WG and Yang J reviewed and edited the manuscript; Yang HT, Zhu JM, Zhang GY, Chen G, Zhang SZ, She YH and Du YY performed the operation; Wang FR, He N, Cui F, Du YY and She YH provided the pictures; Shi YH, Yang J, He N, She YH, Du YY and Zhang GY critically revised the manuscript. All authors have read and approved the final manuscript. Yang HT and Wang FR contributed equally to this work as co-first authors. The reasons for designating Zhu JM and Zhang GY as co-corresponding authors are threefold. Both co-corresponding authors were involved in the conceptualization and writing of the original article, as well as the follow-up of patients. Zhu JM was primarily responsible for the diagnosis and treatment of the patient, while Zhang GY was primarily responsible for the design and writing of the article. The contributions of the two authors are not significantly different.
Supported by Cuiying Scientific and Technological Innovation Program of Lanzhou University Second Hospital, No. CY2022-QN-A13 and No. CY2023-QN-A02; Lanzhou Science and Technology Plan Project, No. 2023-4-26; Gansu Provincial Natural Science Foundation Project, No. 23JRRA1630 and No. 24JRRA331; and Gansu Provincial Health Research Program Project, No. GSWSKY-2019-32.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
https://creativecommons.org/Licenses/by-nc/4.0/ Corresponding author: Geng-Yuan Zhang, MD, Doctor, Department of General Surgery, Lanzhou University Second Hospital, No. 82 Duiyingmen, Chengguan District, Lanzhou 730030, Gansu Province, China.
zgylzdx123456@126.com
Received: June 3, 2024
Revised: August 25, 2024
Accepted: August 28, 2024
Published online: October 27, 2024
Processing time: 116 Days and 3.2 Hours
BACKGROUND
Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm that predominantly affects the kidney and uterus. The occurrence of this tumor in the liver, particularly with simultaneous involvement of the liver and kidney, is exceedingly uncommon. Pathological diagnosis is the gold standard. PEComas usually show positive immunohistochemical staining for melanocytic (HMB-45, Melan-A) and myoid (SMA, muscle-specific actin) markers.
CASE SUMMARY
We presented a noteworthy case of malignant PEComa affecting both the liver and kidney in a 53-year-old man with tuberous sclerosis complex (TSC). FAT2 and TP73 mutations in the kidney were identified and positive expression of diagnostic markers including HMB-45, Melan A, and TFE3 were detected. In addition, we demonstrated that hepatic artery perfusion chemotherapy was ineffective for hepatic PEComa, while surgery remained the most effective approach. Everolimus showed an excellent efficacy in the postoperative treatment of the tumor.
CONCLUSION
Surgical treatment is preferred for malignant PEComa affecting liver and kidney, especially with TSC; everolimus is effective postoperatively.
Core Tip: Perivascular epithelioid cell tumor (PEComa) is rare. Herein, we present the case of a 53-year-old man with the concurrent presence of malignant PEComa in both the liver and kidney, accompanied by tuberous sclerosis complex. During treatment, the hepatic artery perfusion chemotherapy was ineffective for hepatic PEComa, while surgery remained the most effective approach. Everolimus showed an excellent efficacy during the postoperative treatment of the patient. This report provides valuable insights for the diagnosis and treatment of PEComa, paving the way for future clinical practice.
