Anesthesia and perioperative management for giant adrenal Ewing’s sarcoma with inferior vena cava and right atrium tumor thrombus: A case report

BACKGROUND: 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. CASE SUMMARY: 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. CONCLUSION: 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.