Deng CH, Zhou Y, Chen J, He GF, Fu QS, Li JM, Wang G, Hu XD. Diagnostic and therapeutic considerations for primary penal synovial sarcoma with specific MDM2 gene amplification: A case report. World J Clin Urol 2025; 14(1): 104791 [DOI: 10.5410/wjcu.v14.i1.104791]
Corresponding Author of This Article
Xiao-Dong Hu, MD, Department of Urology, The Fourth Affiliated Hospital of Dali University, No. 318 Lucheng South Road, Chuxiong 675000, Yunnan Province, China. 2638522518@qq.com
Research Domain of This Article
Urology & Nephrology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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: http://creativecommons.org/licenses/by-nc/4.0/
Chao-Hua Deng, Yuan Zhou, Xiao-Dong Hu, Department of Urology, The Fourth Affiliated Hospital of Dali University, Chuxiong 675000, Yunnan Province, China
Jing Chen, Department of Nuclear Magnetic Resonance, The Fourth Affiliated Hospital of Dali University, Chuxiong 675000, Yunnan Province, China
Guo-Fu He, Department of Computed Tomography, The Fourth Affiliated Hospital of Dali University, Chuxiong 675000, Yunnan Province, China
Qun-Shan Fu, Department of Pathology, The Fourth Affiliated Hospital of Dali University, Chuxiong 675000, Yunnan Province, China
Jiong-Ming Li, Guang Wang, The Third Ward of Department of Urology, The Second Affiliated Hospital of Kunming Medical University, Kunming 675000, Yunnan Province, China
Co-first authors: Chao-Hua Deng and Yuan Zhou.
Author contributions: Deng CH and Zhou Y undertook the initial writing of the manuscript and were involved in subsequent revisions; Chen J conducted an analysis of the patient's magnetic resonance imaging data and furnished the corresponding images along with their captions; He GF carried out an analysis of the patient's CT data and provided the relevant images and accompanying captions; Fu QS analyzed the patient's pathological section data and supplied the appropriate images and captions; Li JM and Wang G offered professional guidance and valuable suggestions for the improvement of the article; Hu XD revised the initial draft of the paper and took charge of the submission process.
Supported by The Joint Special Project of Universities in Yunnan Province in 2022, No. 2022-ES-530101-207; and The Hospital-level Scientific Research Project of The Fourth Affiliated Hospital of Dali University, No. 2024YJ14.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: All the authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
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: Xiao-Dong Hu, MD, Department of Urology, The Fourth Affiliated Hospital of Dali University, No. 318 Lucheng South Road, Chuxiong 675000, Yunnan Province, China. 2638522518@qq.com
Received: January 6, 2025 Revised: February 23, 2025 Accepted: March 8, 2025 Published online: March 18, 2025 Processing time: 74 Days and 23.8 Hours
Abstract
BACKGROUND
Primary renal synovial sarcoma (PRSS) is extremely rare in clinical practice, and most cases are associated with SYT-SSX gene fusion. The PRSS with specific MDM2 gene amplification has not been reported so far. Therefore, there is no practical experience regarding the clinical, pathological features and diagnosis and treatment plans for patients of this type. This article reports a case of PRSS with specific MDM2 gene amplification.
CASE SUMMARY
The patient was preoperatively diagnosed with a malignant tumor of the left kidney (with a high probability of clear cell carcinoma). During the operation, a radical left nephrectomy was performed. The postoperative pathological examination report confirmed synovial sarcoma, and the gene test suggested PRSS with specific MDM2 gene amplification. Forty-eight days after the operation, the patient presented with "abdominal distension and diarrhea" and was found to have a huge metastatic tumor in the original left renal area. The patient died clinically 17 hours after admission due to "multiple organ failure".
CONCLUSION
PRSS with MDM2 gene amplification has a poorer prognosis, a higher degree of malignancy, and a faster progression, and clinicians need to be highly vigilant.
Core Tip: Primary renal synovial sarcoma (PRSS) is rare, mostly with SYT-SSX gene fusion. This paper reports a case of PRSS with specific MDM2 gene amplification. The patient had metastasis soon after surgery and died rapidly, suggesting this type of PRSS has poor prognosis, high malignancy and rapid progression, which requires clinical vigilance.
