RHABDOMYOSARCOMA IN THE INFERIOR VENA-CAVA WITH SECONDARY BUDD-CHIARISYNDROME

A 63-year-old man with symptoms of obstruction of the inferior vena ca va was examined by computed tomography, ultrasound imaging and angiogr aphy. Examination revealed a tumor in the inferior vena cava, and tran svenous biopsy revealed a rhabdomyosarcoma. The tumor was surgically r esected and was easily separated from the surrounding tissues. Neverth eless, a local recurrence developed 43 days after the operation, and t he patient's condition deteriorated rapidly. Hepatomegaly and ascites believed to represent the Budd-Chiari syndrome were noted. The patient died on the 163rd postoperative day. Autopsy revealed a tumor extendi ng from the inferior vena cava just above the right renal vein to the right atrium and involving the lobus caudatus of the liver. Clinically , the tumor was thought to have arisen from the middle segment of the inferior vena cava. However, a diagnosis of primary hepatic rhabdomyos arcoma with extrahepatic growth could not be excluded. Only 12 cases o f primary liver rhabdomyosarcoma have been reported, and none of those patients demonstrated Budd-Chiari syndrome. Our patient, diagnosed as rhabdomyosarcoma with secondary Budd-Chiari syndrome, is believed to be the first such report.