RSS-Feed abonnieren

DOI: 10.1055/s-0045-1810114
The Diagnostic Quandary of Primary Renal Ewing's Sarcoma on 18F-FDG PET/CT Scan: A Case Report with a Thorough Review of Literature

Abstract
Ewing's sarcoma (EWS) of the kidney is an extremely rare and aggressive malignancy, accounting for less than 1% of renal tumors. This report presents a 42-year-old male who initially presented with hematuria and intermittent fever, with imaging studies suggesting a renal cell carcinoma (RCC). However, histopathological and immunohistochemical analysis, including CD99 positivity and NKX2.2 nuclear expression, confirmed the diagnosis of primary renal EWS. The patient was promptly started on neoadjuvant chemotherapy (VAC + IE [vincristine, adriamycin, cyclophosphamide, ifosfamide, and etoposide]), which he tolerated well without significant adverse effects. Given its nonspecific clinical and 18F-fluorodeoxyglucose positron emission tomography/computed tomography scan presentation, the case highlights the diagnostic challenges in distinguishing renal EWS from more common renal neoplasms such as RCC. Early diagnosis through histopathology and immunohistochemistry is crucial for guiding appropriate management. Given the aggressive nature of renal EWS, a multimodal treatment approach involving chemotherapy followed by radical nephrectomy is essential for improving prognosis. This case sheds light on the importance of considering renal EWS in the differential diagnosis of renal masses and emphasizes the role of early intervention in enhancing survival outcomes.
Keywords
18F-FDG PET/CT scan - Ewing's sarcoma - kidney - renal neoplasms - small round cell sarcomaInformed Consent
Written informed consent was taken from the patient to participate in this study and for the publication of any potentially identifiable images or data included in this article. Ethical review and approval are not required, and a waiver for the publication of case reports is provided as per institutional requirements.
PLACE OF STUDY: Department of Pathology, Vardhman Mahavir Medical College And Safdarjung Hospital, New Delhi – 110029, India.
Publikationsverlauf
Artikel online veröffentlicht:
21. Juli 2025
© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)
Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India
-
References
- 1 Tarek N, Said R, Andersen CR. et al. Primary Ewing sarcoma/primitive neuroectodermal tumor of the kidney: the MD Anderson Cancer Center experience. Cancers (Basel) 2020; 12 (10) 2927
- 2 Tsokos M, Alaggio RD, Dehner LP, Dickman PS. Ewing sarcoma/peripheral primitive neuroectodermal tumor and related tumors. Pediatr Dev Pathol 2012; 15 (1, Suppl): 108-126
- 3 Braaten M, Braaten J, Chaddha J. et al. Survival and treatment in older patients with Ewing sarcoma: an analysis of the national cancer database. Eur J Med Res 2024; 29 (01) 29
- 4 Zhang S, Li Y, Wang R, Song B. Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a case report and literature review. Transl Androl Urol 2019; 8 (05) 562-566
- 5 Singh MM, Verma S, Kakkar L, Deswal S, Husain N. Ewing's sarcoma of the right kidney with multiple metastasis on PET/CT. Egypt J Radiol Nucl Med 2023; 54: 2
- 6 Bontoux C, Khaddour S, Pérot G. et al. Case report of an Ewing's sarcoma/primitive neuroectodermal tumor of the kidney. Int Urol Nephrol 2018; 50 (08) 1449-1451
- 7 Risi E, Iacovelli R, Altavilla A. et al. Clinical and pathological features of primary neuroectodermal tumor/Ewing sarcoma of the kidney. Urology 2013; 82 (02) 382-386
- 8 Ellinger J, Bastian PJ, Hauser S, Biermann K, Müller SC. Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies. Urology 2006; 68 (02) 257-262
- 9 Sadiq M, Ahmad I, Shuja J, Ahmad K. Primary Ewing sarcoma of the kidney: a case report and treatment review. CEN Case Rep 2017; 6 (02) 132-135
- 10 Rangankar V, Dhande A, Khaladkar SM, Kirdat Patil PP, Bhuibhar G. Primary Ewing's sarcoma of the kidney mimicking renal cell carcinoma with widespread metastasis: a case report and a brief review of the literature. Cureus 2024; 16 (07) e64896
- 11 Senkhum O, Attawettayanon W, Pripatnanont C, Chalieopanyarwong V. A primary Ewing's sarcoma of the kidney: a case report and review of literature. Urol Ann 2023; 15 (03) 334-336
- 12 İlhan Y, Suntur C, Toru HS. et al. Primary Ewing sarcoma of the kidney in a 54-year-old male: a rare case report. EJMA 2023; 3 (01) 40-43
- 13 Choubey S, Pipara G, Kumar A. Ewing's sarcoma of the kidney: a rare entity. World J Nephrol Urol 2017; 6 (1–2): 18-20
- 14 Shibuya R, Matsuyama A, Nakamoto M, Shiba E, Kasai T, Hisaoka M. The combination of CD99 and NKX2.2, a transcriptional target of EWSR1-FLI1, is highly specific for the diagnosis of Ewing sarcoma. Virchows Arch 2014; 465 (05) 599-605
- 15 Khudair AD, Khudair AD, Al-Rawahia T. et al. Unveiling the uncommon: a case of metastatic Ewing sarcoma of the kidney. Cureus 2024; 16 (01) e52970
- 16 Yu H, Ge Y, Guo L, Huang L. Potential approaches to the treatment of Ewing's sarcoma. Oncotarget 2017; 8 (03) 5523-5539
- 17 Kairouani M, Mokrim M, Mellas N. et al. Metastatic Ewing's sarcoma/PNET of kidney in 40 year old patient. Int J Surg Case Rep 2012; 3 (06) 215-217
- 18 Kratochwil C, Flechsig P, Lindner T. et al. 68Ga-FAPI PET/CT: tracer uptake in 28 different kinds of cancer. J Nucl Med 2019; 60 (06) 801-805
- 19 Choudhury ARC, Jain SG, Reghunath A, Ghasi RG, Kaur N, Kolte S. Primary Ewing's sarcoma of the kidney: a rare masquerader of renal cell carcinoma on imaging. Egypt J Radiol Nucl Med 2022; 53: 53
- 20 Cheng L, Xu Y, Song H, Huang H, Zhuo D. A rare entity of primary Ewing sarcoma in kidney. BMC Surg 2020; 20 (01) 280
- 21 Alghamdi MHA, Alawad SA, Alharbi MG, Alabdulsalam AK, Almodhen F, Alasker A. A rare case of Ewing's sarcoma of the kidney. Urol Case Rep 2019; 29: 101094
- 22 Almeida MF, Patnana M, Korivi BR, Kalhor N, Marcal L. Ewing sarcoma of the kidney: a rare entity. Case Rep Radiol 2014; 2014: 283902