Advanced Adrenocortical Carcinoma with Vena Caval Tumor Thrombus Treated with Extended Surgery and Subsequent Chemotherapy

A 36-year-old female was referred to our hospital for a giant abdominal mass found by ultrasound examination. A computed tomographic scan showed a large retroperitoneal mass measuring 11 cm in diameter suspected to be liver invasion, a right atrial and inferior vena cava (IVC) tumor thrombus with obstruction of hepatic vein junction of IVC, and small lung metastases. She was diagnosed with cT4N0M1 adrenocortical carcinoma (ACC) by a needle biopsy and radiographic examination. Right adrenalectomy and thrombectomy were successfully performed without cardiac arrest and without liver dissection. The operative time was 485 minutes, and the estimated blood loss was 7, 533 ml. No major peri- or postoperative complications were observed. For the residual lung mass, a first line combination chemotherapy with etoposide, doxorubicin, cisplatin and mitotane followed by a second line chemotherapy with gemcitabine and capecitabine were administered. She has been alive with disease for 45 months under mitotane treatment against residual lung metastases. In conclusion, extended surgery could be successfully performed for advanced ACC with right atrium and IVC tumor thrombus. Although careful planning is needed for successful surgery, combination therapy with extended surgery and subsequent systematic chemotherapy may provide a substantial benefit in patients with advanced ACC