Editorial
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. May 14, 2024; 30(18): 2374-2378
Published online May 14, 2024. doi: 10.3748/wjg.v30.i18.2374
Hepatic perivascular epithelioid cell tumors: Benign, malignant, and uncertain malignant potential
Marcelo Fabián Amante
Marcelo Fabián Amante, División Patología, Hospital General de Agudos Cosme Argerich, Buenos Aires C1155AHA, Argentina
Marcelo Fabián Amante, División Patología, Hospital Alemán de Buenos Aires, Buenos Aires C1155AHA, Argentina
Author contributions: Amante MF designed the report, reviewed the existing literature, analyzed the data, and wrote the paper.
Conflict-of-interest statement: The author reports no relevant conflicts of interest for this article.
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: http://creativecommons.org/licenses/by-nc/4.0/.
Corresponding author: Marcelo Fabián Amante, MD, Chief Doctor, División Patología, Hospital General de Agudos Cosme Argerich, Pi y Margall 480, 13H, Buenos Aires C1155AHA, Argentina. marcelofabianamante@gmail.com
Received: December 24, 2023
Revised: February 20, 2024
Accepted: April 25, 2024
Published online: May 14, 2024
Processing time: 139 Days and 10.2 Hours
Abstract

In 2013, the World Health Organization defined perivascular epithelioid cell tumor (PEComa) as “a mesenchymal tumor which shows a local association with vessel walls and usually expresses melanocyte and smooth muscle markers.” This generic definition seems to better fit the PEComa family, which includes angiomyolipoma, clear cell sugar tumor of the lung, lymphangioleiomyomatosis, and a group of histologically and immunophenotypically similar tumors that include primary extrapulmonary sugar tumor and clear cell myomelanocytic tumor. Clear cell tumors with this immunophenotypic pattern have also had their malignant variants described. When localizing to the liver, preoperative radiological diagnosis has proven to be very difficult, and most patients have been diagnosed with hepatocellular carcinoma, focal nodular hyperplasia, hemangioma, or hepatic adenoma based on imaging findings. Examples of a malignant variant of the liver have been described. Finally, reports of malignant variants of these lesions have increased in recent years. Therefore, we support the use of the Folpe criteria, which in 2005 established the criteria for categorizing a PEComa as benign, malignant, or of uncertain malignant potential. Although they are not considered ideal, they currently seem to be the best approach and could be used for the categorization of liver tumors.

Keywords: PEComas; Liver; Pathology; Malignant; Benign; Uncertain malignant potential

Core Tip: Perivascular epithelioid cell tumors are rare mesenchymal tumors for which the diagnostic criterion appears to be exclusively histopathological. They are characterized by a proliferation of perivascular epithelioid cells and the coexpression of markers for smooth muscle and melanocytes. They mainly affect females and occur in almost all organs. The incidence of benign and malignant perivascular epithelioid cell tumor of the liver is much lower than in other organs. It is necessary to reinforce the notion that there are malignant variants located in the liver, which are more frequently described along with their histologic and immunohistochemical features.