Retrospective Study
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Sep 21, 2021; 27(35): 5967-5977
Published online Sep 21, 2021. doi: 10.3748/wjg.v27.i35.5967
Hepatic perivascular epithelioid cell tumor: Clinicopathological analysis of 26 cases with emphasis on disease management and prognosis
Shan Zhang, Pan-Pan Yang, Yu-Chen Huang, Hong-Chun Chen, De-Li Chen, Wen-Tian Yan, Ning-Ning Yang, Yuan Li, Nan Li, Zhen-Zhong Feng
Shan Zhang, Department of Pathology, The Second People’s Hospital of Hefei, Hefei 230011, Anhui Province, China
Pan-Pan Yang, Zhen-Zhong Feng, Department of Pathology, The Second Affiliated Hospital of Anhui Medical University, Hefei 230601, Anhui Province, China
Yu-Chen Huang, Hong-Chun Chen, Wen-Tian Yan, Ning-Ning Yang, Nan Li, Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu 233000, Anhui Province, China
De-Li Chen, Department of Gastrointestinal Surgery, The First Affiliated Hospital of Bengbu Medical College, Bengbu 233000, Anhui Province, China
Yuan Li, Department of Pathology, The First Affiliated Hospital of University of Science and Technology of China, Anhui Provincial Hospital, Hefei 230001, Anhui Province, China
Author contributions: Zhang S and Feng ZZ designed the study; Zhang S and Yang PP performed the experiments; Li Y prepared the slices; Zhang S, Huang YC, Chen HC, Yan WT, and Yang NN collected and analyzed the data; Feng ZZ and Li N reviewed the histopathology and IHC results; Chen DL provided critical comments on the manuscript; Zhang S, Yang PP, and Feng ZZ wrote the manuscript; all authors read and approved the final manuscript.
Supported by the Anhui Provincial Natural Science Foundation, No. 1908085MH275; Bengbu Medical College Key projects of Natural Science Foundation, No. BYKF1710; and Bengbu City-Bengbu Medical College Joint Science and Technology Project, No. BYLK201812.
Institutional review board statement: This study was approved by the Ethics and Research Committees of the First Affiliated Hospital of Bengbu Medical College (Anhui Province, China).
Informed consent statement: Informed written consent was obtained from all the patients.
Conflict-of-interest statement: The authors declare no conflicts of interest for this manuscript.
Data sharing statement: No additional data are available.
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:
Corresponding author: Zhen-Zhong Feng, PhD, Professor, Department of Pathology, The Second Affiliated Hospital of Anhui Medical University, No. 678 Furong Road, Hefei 230601, Anhui Province, China.
Received: March 29, 2021
Peer-review started: March 29, 2021
First decision: May 28, 2021
Revised: May 29, 2021
Accepted: August 13, 2021
Article in press: August 13, 2021
Published online: September 21, 2021

Perivascular epithelioid cell tumor (PEComa) is an uncommon tumor of mesenchymal origin. Cases of PEComa in the liver are extremely rare.


To analyze the clinicopathological features and treatment of hepatic PEComa and to evaluate the prognosis after different treatments.


Clinical and pathological data of 26 patients with hepatic PEComa were collected. All cases were analyzed by immunohistochemistry and clinical follow-up.


This study included 17 females and 9 males, with a median age of 50 years. Lesions were located in the left hepatic lobe in 13 cases, in the right lobe in 11, and in the caudate lobe in 2. The median tumor diameter was 6.5 cm. Light microscopy revealed that the tumor cells were mainly composed of epithelioid cells. The cytoplasm contained heterogeneous eosinophilic granules. There were thick-walled blood vessels, around which tumor cells were radially arranged. Immunohistochemical analysis of pigment-derived and myogenic markers in PEComas revealed that 25 cases were HMB45 (+), 23 were Melan-A (+), and 22 SMA (+). TFE3 and Desmin were negative in all cases. All the fluorescence in situ hybridization samples were negative for TFE3 gene break-apart probe. Tumor tissues were collected by extended hepatic lobe resection or simple hepatic tumor resection as the main treatments. Median follow-up was 62.5 mo. None of the patients had metastasis or recurrence, and there were no deaths due to the disease.


Hepatic PEComa highly expresses melanin and smooth muscle markers, and generally exhibits an inert biological behavior. The prognosis after extended hepatic lobe resection and simple hepatic tumor resection is semblable.

Keywords: Hepatic tumor, Perivascular epithelioid cells, PEComa, Immunohistochemistry, Treatment, Prognosis

Core Tip: Hepatic perivascular epithelioid cell tumor (PEComa) exhibits an inert biological behavior, and its diagnosis, treatment, and follow-up are challenging. Our study revealed that there was no difference in the prognosis between simple resection of liver tumor and extended resection of liver lobe. Optimal surgical resection currently is the best treatment option, and radiotherapy, chemotherapy, and immunotherapy may become more effective in future. The number of cases in the current retrospective study was limited by the rarity of hepatic PEComa. Therefore, further multicenter, larger-cohort studies are warranted to investigate the clinicopathological features and biological behavior of hepatic PEComa.