Case Report
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jan 16, 2023; 11(2): 426-433
Published online Jan 16, 2023. doi: 10.12998/wjcc.v11.i2.426
Perivascular epithelioid cell tumors of the liver misdiagnosed as hepatocellular carcinoma: Three case reports
Yan-Qi Kou, Yu-Ping Yang, Wei-Xiang Ye, Wei-Nan Yuan, Shen-Shen Du, Biao Nie
Yan-Qi Kou, Yu-Ping Yang, Wei-Nan Yuan, Shen-Shen Du, Biao Nie, Department of Gastroenterology, The First Affiliated Hospital of Jinan University, Guangzhou 510630, Guangdong Province, China
Wei-Xiang Ye, Department of Gastrointestinal Endoscopy, The First Affiliated Hospital of Jinan University, Guangzhou 510630, Guangdong Province, China
Author contributions: Kou YQ, Yang YP, and Ye WX contributed equally to this manuscript and can be regarded as co-first authors; Nie B and Kou YQ contributed to the conceptualization and methodology; Yang YP, Ye WX, and Kou YQ contributed to the visualization and investigation; Ye WX, Yuan WN and Du SS contributed to the data curation; Yang YP and Kou YQ contributed to the writing and original draft preparation.
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: Biao Nie, MD, PhD, Chief Doctor, Professor, Department of Gastroenterology, The First Affiliated Hospital of Jinan University, No. 613 Huangpu Avenue West, Tianhe District, Guangzhou 510630, Guangdong Province, China. niebiao1974@163.com
Received: September 22, 2022
Peer-review started: September 22, 2022
First decision: November 6, 2022
Revised: November 16, 2022
Accepted: December 15, 2022
Article in press: December 15, 2022
Published online: January 16, 2023
Processing time: 111 Days and 19.4 Hours
Abstract
BACKGROUND

Hepatic perivascular epithelioid cell neoplasms (PEComas) are rare. Diagnostic and treatment experience with hepatic PEComa remains insufficient.

CASE SUMMARY

Three hepatic PEComa cases are reported in this paper: One case of primary malignant hepatic PEComa, one case of benign hepatic PEComa, and one case of hepatic PEComa with an ovarian mature cystic teratoma. During preoperative imaging and pathological assessment of intraoperative frozen samples, patients were diagnosed with hepatocellular carcinoma (HCC), while postoperative pathology and immunohistochemistry subsequently revealed hepatic PEComa. Patients with hepatic PEComa which is misdiagnosed as HCC often require a wider surgical resection. It is easy to mistake them for distant metastases of hepatic PEComa and misdiagnosed as HCC, especially when it's combined with tumors in other organs. Three patients eventually underwent partial hepatectomy. After 1-4 years of follow-up, none of the patients experienced recurrence or metastases.

CONCLUSION

A clear preoperative diagnosis of hepatic PEComa can reduce the scope of resection and prevent unnecessary injuries during surgery.

Keywords: Perivascular epithelioid cell neoplasms; Hepatocellular carcinoma; Case report; Diagnoses; Coexistence

Core Tip: Herein, we present three cases of hepatic perivascular epithelioid cell neoplasms (PEComas): One case of primary malignant PEComa, one case of benign PEComa, and one case of PEComa with ovarian mature cystic teratoma. The first case of PEComa cooccurred with an ovarian mature cystic teratoma. All three cases were misdiagnosed as liver cancer before surgery. A high rate of misdiagnosis of hepatocellular carcinoma is noted among patients with PEComa. A clear preoperative diagnosis of hepatic PEComa is crucial before deciding on a treatment plan, especially with the extent of surgical treatment in hepatocellular carcinoma.