Systematic Reviews
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 6, 2023; 11(28): 6782-6791
Published online Oct 6, 2023. doi: 10.12998/wjcc.v11.i28.6782
Primary adrenal Ewing sarcoma: A systematic review of the literature
Dimitrios K Manatakis, Ioannis Tsouknidas, Emmanouil Mylonakis, Nikolaos Tasis, Maria Ioanna Antonopoulou, Vasileios Acheimastos, Aikaterini Mastoropoulou, Dimitrios P Korkolis
Dimitrios K Manatakis, Emmanouil Mylonakis, Nikolaos Tasis, Vasileios Acheimastos, 2nd Department of Surgery, Athens Naval and Veterans Hospital, Athens 11521, Greece
Ioannis Tsouknidas, General Surgery, Lankenau Medical Center, Main Line Health, Wynnewood, PA 19096, United States
Nikolaos Tasis, Dimitrios P Korkolis, Department of Surgical Oncology, St Savvas Cancer Hospital, Athens 11522, Greece
Maria Ioanna Antonopoulou, Department of Surgery, Naval Hospital of Crete, Chania 73200, Greece
Aikaterini Mastoropoulou, Department of Pediatrics, Stony Brook University Hospital, Stony Brook, NY 11794, United States
Author contributions: Manatakis DK and Tasis NP designed the study; Antonopoulou MI, Acheimastos V and Mylonakis E performed the research; Mylonakis E, Tsouknidas I and Mastoropoulou A wrote and revised the manuscript; Manatakis DK and Korkolis DP coordinated the project; All authors have read and approved the final manuscript.
Conflict-of-interest statement: The authors deny any conflict of interest.
PRISMA 2020 Checklist statement: The authors have read the PRISMA 2020 Checklist, and the manuscript was prepared and revised according to the PRISMA 2020 Checklist.
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: Ioannis Tsouknidas, MD, MSc, Research Fellow, Surgeon, General Surgery, Lankenau Medical Center, Main Line Health, No. 100E Lancaster Ave, Wynnewood, PA 19096, United States. tsouknidasioannis@gmail.com
Received: April 20, 2023
Peer-review started: April 20, 2023
First decision: June 12, 2023
Revised: July 18, 2023
Accepted: September 12, 2023
Article in press: September 12, 2023
Published online: October 6, 2023
Processing time: 157 Days and 11.4 Hours
Core Tip

Core Tip: Primary adrenal Ewing sarcoma (ES) is uncommon with 66 cases reported in the literature since 1989. Patients usually present within the first year from the initiation of symptoms, most frequently complaining of abdominal/flank pain or discomfort, followed by a palpable mass. Open adrenalectomy is the procedure of choice, while minimally invasive techniques are anticipated to be performed more frequently in the future. Regardless of the technique, surgical resection is achieved in the vast majority of cases. The significant progress in molecular biology and genetics of ES over the past decade does not reflect on patient outcomes. ES remains an aggressive tumor with a poor prognosis and high mortality.