Extrarenal Angiomyolipoma Originating from the Right Ovarian Vein Occluding the Inferior Vena Cava and Right Atrium

Abstract

A 68-year-old woman was referred with the incidental sonographic finding of a retroperitoneal solid tumor inside the retrohepatic vena cava extending proximally toward the right atrium. She had nonspecific and mild clinical symptoms such as exertional dyspnea and moderate leg edema. A thoracic and abdominal CT showed a cone-like tumor occluding the suprarenal inferior vena cava and more than 50% of the right atrium. It extended outside the infrarenal vena cava between the vena cava and right kidney (A, B). Although CT did not reveal information about the nature of the process, the primary presumption was a leiomyosarcoma. Because the tumor appeared resectable, no biopsy was performed and resection was scheduled. Through a median sternotomy and laparotomy with right transverse epigastric extension, no peritoneal spread or additional tumor was seen. The right liver, duodenum, and right hemicolon were mobilized from the retroperitoneum, and the pericardium opened to expose the whole tumor. In the retroperitoneum the tumor appeared cone-like and seemed to grow inside the right ovarian vein, slightly displacing the right ureter without infiltration, and extending to the anterior aspect of the vena cava where it entered just below the renal veins. After initiation of cardiopulmonary bypass, the hepatoduodenal ligament, renal veins, and infrarenal cava were clamped, and the right atrium and inferior vena cava were completely opened longitudinally. The capsulated tumor, which was partly adherent to the inner vessel wall, was