Wilms' tumor with vena caval, atrial, and middle hepatic vein tumor thrombus

A 3-year-old male with a right-sided Wilms' tumor presented with tender hep atomegaly and bilateral lower-limb edema. Ultrasound and echocardiography s howed a massive tumor thrombus completely occluding the inferior vena cava, right atrial cavity, and extending retrogradely into the middle hepatic ve in. Two courses of preoperative chemotherapy (Vincristine, actinomycin D, a driamycin) caused minimal shrinkage of the thrombus. The tumor and thrombus were successfully removed with the patient under cardiopulmonary bypass an d deep hypothermic circulatory arrest followed by multiagent chemotherapy ( vincristine, actinomycin D, adriamycin, cyclophosphamide). The child is ali ve and well with no evidence of disease 15 months later. Occlusion of the h epatic vein by a tumor thrombus in Wilms' tumor is a very rare event. It wa s completely removed by the right atrial route under direct vision in this child.