本文报道一例腹膜后尤文肉瘤合并肾静脉及下腔静脉癌栓的病例。患者因"左侧腹痛1月"入院。PET-CT检查提示：左侧肾上腺区见巨大肿块；左侧肾上腺显示不清，肿瘤与胃底、胰腺尾部及左肾上极分界不清；左肾静脉及下腔静脉内考虑为癌栓形成；右肺动脉主干、右中肺及右下肺动脉考虑肺动脉癌栓。经南方医院多学科癌栓团队会诊后行胸腹联合左侧腹膜后肿物切除+肾静脉下腔静脉癌栓取出术+肺动脉癌栓取出术，术后病理结果提示尤文肉瘤。经广东省医学会泌尿外科疑难病例多学科会诊，最终确定术后先行辅助化疗，方案为VDC/IE（长春新碱+多柔比星+环磷酰胺与异环磷酰胺+依托泊苷交替）。腹膜后占位合并肾静脉及下腔静脉癌栓手术难度大风险高，癌栓脱落至肺动脉等处可引起严重并发症。此类患者术前需多学科讨论，术前做好充分准备，术中需多学科协作，术后需个体化辅助治疗。

This article reports a case of posterior left retroperitoneal Ewing sarcoma complicated by renal vein and inferior vena cava tumor thrombus.The patient was admitted due to "left-sided abdominal pain for one month". PET-CT scan revealed a large mass in the left adrenal region, the left adrenal gland was indistinct, and the tumor boundaries with the gastric fundus, pancreatic tail, and upper pole of the left kidney were unclear; tumor thrombus formation was suspected in the left renal vein and inferior vena cava, pulmonary artery tumor thrombus was suspected in the main trunk of the right pulmonary artery, right middle pulmonary artery, and right lower pulmonary artery. After discussion by the multidisciplinary team at Nanfang Hospital, Southern Medical University, a combined thoracoabdominal left retroperitoneal mass resection, renal vein and inferior vena cava tumor thrombus removal, and pulmonary artery tumor thrombus removal were performed. Postoperative pathology results indicated Ewing sarcoma. After a multidisciplinary consultation of difficult cases in Guangdong Urological Association, It was ultimately determined to administer adjuvant chemotherapy first postoperatively, with a regimen of VDC/IE (vincristine + doxorubicin + cyclophosphamide alternating with ifosfamide + etoposide). Retroperitoneal masses complicated by renal vein and inferior vena cava tumor thrombus present significant surgical challenges and high risks, as tumor thrombus detachment can lead to serious complications such as pulmonary artery embolism. These patients require multidisciplinary discussion before surgery, thorough preoperative preparation, multidisciplinary collaboration during surgery, and individualized postoperative adjuvant treatment.