Das Suvraraj, Aggarwal Gaurav, Gupta Sujoy, Midha Divya
Surgical Oncology, Tata Medical Center, Newtown, Kolkata, West Bengal, India.
Department of Uro-Oncology, Tata Medical Center, Newtown, Kolkata, West Bengal, India.
Innov Surg Sci. 2021 Jan 7;6(1):20200022. doi: 10.1515/iss-2020-0022. eCollection 2021 Jan.
Extraskeletal Ewing's sarcoma is a rarity, with a renal primary in an adult, being even rarer. There is no consensus on the optimal imaging modality, as well as best therapeutic option, making them an enigma for clinicians.
We report the case of a 34-year-old lady, a known case of invasive lobular carcinoma of the left breast (ER,PR positive, Her2neu negative), having completed treatment in 2017, wherein, on an ultrasound evaluation for left flank pain, was incidentally found to have a left renal mass. A CT scan corroborated with the ultrasound, with an additional Level 1, left renal vein thrombus. She underwent an open left radical nephrectomy with renal vein thrombectomy. Histopathology of the resected tumor revealed features of Ewing's sarcoma of the kidney, confirmed by Fluorescent In Situ Hybridisation (FISH) and Immunohistochemistry (IHC).
Primary renal Ewing's sarcoma in an adult is a rare occurrence, with no characteristic imaging features, and no universally accepted guideline based management protocols. Akin with standard Ewings sarcoma treatment strategies, a margin negative- radical nephrectomy with adjuvant chemotherapy, seems the most apt treatment strategy.
骨外尤文肉瘤较为罕见，成人原发性肾尤文肉瘤更是罕见。对于最佳影像学检查方式以及最佳治疗方案尚无共识，这使其成为临床医生的难题。
我们报告一例34岁女性病例，该患者为已知的左乳浸润性小叶癌（雌激素受体、孕激素受体阳性，人表皮生长因子受体2阴性），于2017年完成治疗，此次因左侧胁腹疼痛行超声检查时，偶然发现左肾肿物。CT扫描结果与超声检查相符，还发现左侧肾静脉一级血栓形成。她接受了开放性左肾根治性切除术及肾静脉血栓切除术。切除肿瘤的组织病理学检查显示为肾尤文肉瘤特征，荧光原位杂交（FISH）和免疫组织化学（IHC）证实了这一诊断。
成人原发性肾尤文肉瘤罕见，无特征性影像学表现，也没有普遍接受的基于指南的管理方案。与标准尤文肉瘤治疗策略类似，切缘阴性的根治性肾切除术联合辅助化疗似乎是最合适的治疗策略。
