Wang Ji-Lian, Xu Chuan-Ya, Geng Chun-Jing, Liu Lei, Zhang Ming-Zhu, Wang Hua, Xiao Ruo-Tao, Liu Lu, Zhang Geng, Ni Cheng, Guo Xiang-Yang
Department of Anesthesiology, Peking University Third Hospital, Beijing 100191, China.
Department of Urology, Peking University Third Hospital, Beijing 100191, China.
World J Clin Cases. 2022 Jan 14;10(2):643-655. doi: 10.12998/wjcc.v10.i2.643.
Ewing's sarcoma of the adrenal gland with inferior vena cava (IVC) and right atrium thrombus is extremely rare. Here, we report a case of giant adrenal Ewing's sarcoma with IVC and right atrium tumor thrombus and summarize the anesthesia and perioperative management.
A young female was admitted to the Department of Urology with intermittent pain under the right costal arch for four months. Enhanced abdominal computed tomography revealed a large retroperitoneal mass (22 cm in diameter), which may have originated from the right adrenal gland and was closely related to the liver. Transthoracic echocardiography showed a strong echogenic filling measuring 70 mm extended from the IVC into the right atrium and ventricle. After preoperative preparation with cardiopulmonary bypass, sufficient blood products, transesophageal echocardiography and multiple monitoring, tumor and thrombus resection by IVC exploration and right atriotomy were successfully performed by a multidisciplinary team. Intraoperative hemodynamic stability was the major concern of anesthesiologists and the status of tumor thrombus and pulmonary embolism were monitored continuously. During transfer of the patient to the intensive care unit (ICU), cardiac arrest occurred without external stimulus. Cardiopulmonary resuscitation was performed immediately and cardiac function was restored after 1 min. In the ICU, extracorporeal membrane oxygenation (ECMO) and continuous renal replacement therapy (CRRT) were provided to maintain cardiac, liver and kidney function. Histopathologic examination confirmed the diagnosis of Ewing's sarcoma. After postoperative treatments and rehabilitation, the patient was discharged from the urology ward.
An adrenal Ewing's sarcoma with IVC and right atrium thrombus is extremely rare, and its anesthesia and perioperative management have not been reported. Thus, this report provides significant insights in the perioperative management of patients with adrenal Ewing's sarcoma and IVC tumor thrombus. Intraoperative circulation fluctuations and sudden cardiovascular events are the major challenges during surgery. In addition, postoperative treatments including ECMO and CRRT provide essential support in critically ill patients. Moreover, this case report also highlights the importance of multidisciplinary cooperation during treatment of the disease.
肾上腺尤因肉瘤合并下腔静脉（IVC）及右心房血栓极为罕见。在此，我们报告一例巨大肾上腺尤因肉瘤合并IVC及右心房肿瘤血栓的病例，并总结麻醉及围手术期管理经验。
一名年轻女性因右肋弓下间歇性疼痛4个月入住泌尿外科。增强腹部计算机断层扫描显示一个巨大的腹膜后肿块（直径22厘米），可能起源于右肾上腺，且与肝脏关系密切。经胸超声心动图显示一个从IVC延伸至右心房和心室的70毫米强回声充盈物。在进行体外循环、充足血液制品、经食管超声心动图及多项监测等术前准备后，一个多学科团队成功通过IVC探查及右心房切开术切除肿瘤及血栓。术中血流动力学稳定是麻醉医生主要关注的问题，同时持续监测肿瘤血栓及肺栓塞情况。在将患者转运至重症监护病房（ICU）期间，患者无外界刺激下发生心脏骤停。立即进行心肺复苏，1分钟后心脏功能恢复。在ICU，给予体外膜肺氧合（ECMO）及持续肾脏替代治疗（CRRT）以维持心、肝、肾功能。组织病理学检查确诊为尤因肉瘤。经过术后治疗及康复，患者从泌尿外科病房出院。
肾上腺尤因肉瘤合并IVC及右心房血栓极为罕见，其麻醉及围手术期管理此前未见报道。因此，本报告为肾上腺尤因肉瘤合并IVC肿瘤血栓患者的围手术期管理提供了重要见解。术中循环波动及突发心血管事件是手术期间的主要挑战。此外，包括ECMO及CRRT在内的术后治疗为重症患者提供了必要支持。而且，本病例报告还突出了该疾病治疗过程中多学科合作的重要性。
