Published online Oct 6, 2023. doi: 10.12998/wjcc.v11.i28.6782
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
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.
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.
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.
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.
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%.
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.
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.