Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. May 6, 2022; 10(13): 4273-4279
Published online May 6, 2022. doi: 10.12998/wjcc.v10.i13.4273
Hepatic perivascular epithelioid cell tumor: A case report
Yong-Fang Li, Liang Wang, Yi-Jing Xie
Yong-Fang Li, Liang Wang, Department of Hepatology, Lanzhou University The Second Hospital, Lanzhou 730030, Gansu Province, China
Yi-Jing Xie, Department of Radiology, Lanzhou University The Second Hospital, Lanzhou 730030, Gansu Province, China
Author contributions: Li YF was the patient’s physician, collected case information, reviewed the literature and contributed to manuscript drafting; Xie YJ analyzed and interpreted the imaging findings; Wang L was responsible for the revision of the manuscript for important intellectual content; all authors issued final approval for the version to be submitted.
Supported by Gansu Provincial Natural Science Foundation, No. 21JR7RA417; Lanzhou Science and Technology Development guiding Plan Project, No. 2019-ZD-72.
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 declare that they have no conflict 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: Liang Wang, MM, Department of Hepatology, Lanzhou University The Second Hospital, No. 82 Cuiyingmen, Chengguan District, Lanzhou 730030, Gansu Province, China. wangliang@lzu.edu.cn
Received: October 10, 2021
Peer-review started: October 10, 2021
First decision: December 10, 2021
Revised: December 18, 2021
Accepted: March 15, 2022
Article in press: March 15, 2022
Published online: May 6, 2022
Processing time: 202 Days and 0.3 Hours
Abstract
BACKGROUND

Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor with histologic and immunophenotypic characteristics of perivascular epithelioid cells, has a low incidence, and can involve multiple organs. PEComa originating in the liver is extremely rare, with most cases being benign, and only a few cases are malignant. Good outcomes are achieved with radical surgical resection, but there is no effective treatment for some large tumors and specific locations that are contraindicated for surgery.

CASE SUMMARY

A 32-year-old woman was admitted to our hospital with a palpable abdominal mass and progressive deterioration since the previous month. An ultrasound-guided percutaneous liver aspiration biopsy was performed. Postoperative pathological immunohistochemical staining was HMB45, Melan-A, and smooth muscle actin positive. Perivascular epithelioid tumor was diagnosed. The tumor was large and could not be completely resected by surgery. Further digital subtraction angiography revealed a rich tumor blood supply, and interventional embolization followed by surgery was recommended. Finally, the patient underwent transarterial embolization (TAE) combined with sorafenib for four cycles. Angiography reexamination indicated no clear vascular staining of the tumor, and the tumor had shrunk. The patient was followed up for a short period of time, achieved a stable condition, and surgery was recommended.

CONCLUSION

Adjuvant combination treatment with TAE and sorafenib is safe and feasible as it shrinks the tumor preoperatively and facilitates surgery.

Keywords: Perivascular epithelioid cell tumor; Liver; Treatment; Transarterial embolization; Sorafenib; Case report

Core Tip: Transarterial embolization in combination with sorafenib is a targeted anti-angiogenic therapy that is widely used in the palliative treatment of unresectable hepatocellular carcinoma. However, this combination therapy has not been reported in perivascular epithelioid cell tumor (PEComa). In patients with PEcoma of the liver that cannot be surgically resected or when surgery is contraindicated, this combination of adjuvant therapy is safe and feasible to shrink the tumor and allow the patient to undergo surgery.