Khajeh-Mehrizi Ahmad, Karimi Mehdi, Mortezazadeh Masoud, Zarei Hamidreza, Haghshenas Hoda, Nili Fatemeh
Department of Hematology-Oncology, Cancer Institute, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences (TUMS), Tehran, Iran.
Faculty of Medicine, Bogomolets National Medical University (NMU), Kyiv, Ukraine.
J Med Case Rep. 2025 Mar 18;19(1):122. doi: 10.1186/s13256-025-05157-7.
Renal-origin Ewing's sarcoma is an extremely rare malignancy often misdiagnosed owing to its nonspecific presentation and similarities to other renal tumors. Accurate diagnosis requires a combination of clinical evaluation and advanced diagnostic techniques. Limited case reports make understanding its clinical course and management challenging. This case report aims to raise awareness of renal-origin Ewing's sarcoma, highlight diagnostic challenges, and discuss effective multidisciplinary management strategies to improve patient outcomes.
A 32-year-old Iranian female patient presented with a chief complaint of progressive dyspnea, anorexia, and night sweating in the last 2 weeks before her admission. Computed tomography scan showed a tumoral lesion in the left kidney with thrombosis extending into the left renal vein and inferior vena cava up to the right atrium. The patient underwent open cardiac surgery and a radical nephrectomy. During surgery, the mass protruded from the inferior vena cava into the right atrium; it was ultimately diagnosed as renal-origin Ewing's sarcoma, and EWSR1 rearrangement was confirmed on pathology.
This case highlights the importance of a thorough diagnostic approach in patients presenting with a renal mass and the value of a multidisciplinary strategy, combining clinical presentation, imaging, histopathology, immunohistochemistry, and molecular studies to achieve an accurate diagnosis. It underscores the critical need for increased awareness and research into the rare entity of renal-origin Ewing's sarcoma, as clinical and pathological information on this condition is limited. Furthermore, this case emphasizes the necessity of timely diagnosis and tailored management to optimize treatment outcomes and improve survival rates in such rare and challenging presentations.
not applicable.
肾源性尤因肉瘤是一种极为罕见的恶性肿瘤，因其表现不具特异性且与其他肾肿瘤相似，常被误诊。准确诊断需要临床评估与先进诊断技术相结合。有限的病例报告使得了解其临床病程和治疗颇具挑战。本病例报告旨在提高对肾源性尤因肉瘤的认识，突出诊断难题，并探讨有效的多学科管理策略以改善患者预后。
一名32岁的伊朗女性患者入院前两周主诉进行性呼吸困难、厌食和盗汗。计算机断层扫描显示左肾有一个肿瘤性病变，血栓延伸至左肾静脉和下腔静脉直至右心房。患者接受了心脏直视手术和根治性肾切除术。手术中，肿块从下腔静脉突出至右心房；最终诊断为肾源性尤因肉瘤，病理检查证实存在EWSR1重排。
本病例突出了对肾肿块患者进行全面诊断方法的重要性，以及多学科策略的价值，该策略结合临床表现、影像学、组织病理学、免疫组织化学和分子研究以实现准确诊断。它强调了提高对肾源性尤因肉瘤这一罕见实体的认识和研究的迫切需求，因为关于这种疾病的临床和病理信息有限。此外，本病例强调了及时诊断和针对性管理的必要性，以优化治疗结果并提高此类罕见且具有挑战性病例的生存率。
不适用。
