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
ARTICLE HIGHLIGHTS
Research background

Ewing sarcoma (ES) is an aggressive malignant primary osseous tumor, which is commonly observed in the young population. Visceral organs and particularly adrenal glands are rarely impacted.

Research motivation

Therapeutic protocols for the treatment of ES/primitive neuroectodermal tumor (PNET) are not standardized and these tumors are treated according to the soft tissue sarcoma guidelines of the European Society of Medical Oncology (ESMO) and National Comprehensive Cancer Network (NCCN), due to limited knowledge.

Research objectives

The aim of the present study is to present an up-to-date, systematic review of the literature on primary adrenal ES/PNET, with emphasis on diagnosis, therapy and oncological outcomes.

Research methods

A systematic review of the literature was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020 statement. PubMed/MEDLINE, EMBASE and Google Scholar bibliographic databases were searched to identify articles that included patients with ES/PNET of the adrenal gland from 1989 to 2022. Demographic, clinical, pathological and oncological data of patients were analyzed by SPSS version 29.0.

Research results

Fifty-two studies were included in the current systematic review, describing a total of 66 patients. The mean age at diagnosis was 26.4 years and the most frequent complaint was abdominal or flank pain/discomfort. At the time of diagnosis, average tumor size was 11.4 cm. 27.9% of the tumors were metastatic and 23.0% had inferior vena cava or renal vein neoplastic thrombus. Open adrenalectomy was the procedure of choice (80.0% of patients), and a more extensive resection was required in 27.0% of these patients. Immunohistochemistry staining for CD99 was positive in 98.4%, and molecular testing for the translocation EWS RNA binding protein 1-Friend leukemia integration 1 was positive in all the patients tested. Median overall survival was 15 mo and 24-mo overall survival was 40.5%.

Research conclusions

Primary adrenal ES/PNET is a tumor with low prevalence. Diagnosis typically occurs when the tumor has reached an advanced stage. Immunohistochemical staining for CD99 is essential to support the diagnosis of ES/PNET from other small round cell tumors. To date, these tumors are treated according to the soft tissue sarcoma guidelines of ESMO and NCCN, with surgical excision and negative surgical margins being the preferred treatment of choice, when feasible. Unfortunately, the disease has a high recurrence rate, and a relatively low survival rate.

Research perspectives

In the future, minimally invasive techniques will be used more frequently in the surgical treatment of primary Ewing adrenal sarcoma. In addition, research in biomarkers and artificial intelligence science will assist with stratifying patients into groups, and lead to the development of individualized treatments and prognostic treatment-response scores in chemotherapy and/or radiotherapy.