Dong Guo-Fan, Hou Ya-Kun, Ma Qi, Ma Shuang-Yu, Wang Yu-Jie, Rexiati Mulati, Wang Wen-Guang
Department of Urologic Surgery, The First Affiliated Hospital of Xinjiang Medical University, Urumqi 830000, Xinjiang Uygur Autonomous Region, China.
World J Clin Cases. 2024 Aug 16;12(23):5431-5440. doi: 10.12998/wjcc.v12.i23.5431.
Primary renal Ewing's sarcoma (ES) is extremely rare, and only two cases causing Cushing's syndrome (CS) have been reported to date. We report that the case of an 18-year-old patient is diagnosed primary renal ES with typical CS characterized by purple stripes, weight gain, and hypertension.
CS was first diagnosed by laboratory testing. A huge tumor was revealed in the kidney following an imaging examination. Moreover, brain and bone metastases were observed. After comprehensive treatment, primarily based on surgery, primary renal ES was pathologically diagnosed with a typical genetic mutation through genetic testing. Furthermore, the glucocorticoid level returned to normal. By the ninth postoperative month of follow-up, the patient was recovering well. Cushing-related symptoms had improved, and a satisfactory curative effect was achieved.
Primary renal ES, a rare adult malignant tumor, can cause CS and a poor prognosis.
原发性肾尤文肉瘤（ES）极为罕见，迄今为止仅报道过两例导致库欣综合征（CS）的病例。我们报告一例18岁患者，被诊断为原发性肾ES并伴有典型的CS，表现为紫纹、体重增加和高血压。
CS最初通过实验室检查确诊。影像学检查发现肾脏有一个巨大肿瘤。此外，还观察到脑和骨转移。经过以手术为主的综合治疗，通过基因检测对原发性肾ES进行了病理诊断，发现有典型的基因突变。此外，糖皮质激素水平恢复正常。术后随访至第九个月，患者恢复良好。与库欣综合征相关的症状有所改善，取得了满意的疗效。
原发性肾ES是一种罕见的成人恶性肿瘤，可导致CS且预后不良。
