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
Abstract
BACKGROUND

Ewing sarcoma (ES) is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults. The musculoskeletal system is the main body system impacted and ES is rarely seen in the visceral organs particularly the adrenal gland.

AIM

To present a comprehensive review of primary adrenal ES, with emphasis on diagnosis, therapy and oncological outcomes.

METHODS

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

RESULTS

A total of 52 studies were included for review (47 case reports and 5 case series) with 66 patients reported to have primary adrenal ES. Mean age at diagnosis was 26.4 ± 15.4 years (37.9% males, 57.6% females, sex not reported in 3 cases). The most frequent complaint was abdominal/flank pain or discomfort (46.4%) followed by a palpable mass (25.0%), and the average duration of symptoms was 2.6 ± 3.1 mo. The imaging modality of choice was computed tomography scan (81.5%), followed by magnetic resonance imaging (20.4%). Preoperative staging revealed that 17 tumors (27.9%) were metastatic and 14 patients had inferior vena cava or renal vein neoplastic thrombus at initial diagnosis. Open adrenalectomy was performed in the majority of cases (80.0%), of which 27.9% required more extensive resection. Minimally invasive surgery was attempted in 8.2% of tumors. Complete surgical resection was achieved in 89.4% of the patients. Adjuvant therapy was administered to 32 patients, in the form of chemotherapy (62.5%), radiotherapy (3.1%) or combination (34.4%). Median overall survival was 15 mo and 24-mo overall survival was 40.5%. Median disease-free survival was 10 mo and 24-mo disease-free survival was 33.3%.

CONCLUSION

The significant progress in molecular biology and genetics of ES does not reflect on patient outcomes. ES remains an aggressive tumor with a poor prognosis and high mortality.

Keywords: Ewing sarcoma; Primary adrenal tumor; Adrenalectomy

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.