Hepatoblastoma metastatic to the right atrium responding to chemotherapy alone

An 18-month-old boy presented with abdominal pain and distension. On physical examination there was a 10 x 7-cm mass in the right upper abdominal quadrant. His alpha-fetoprotein level was 175,000 IU/mL. Abdominal magnetic resonance findings revealed hepatomegaly with multiple tumor masses involving nearly all the segments of the liver (PRETEXT IV). The tumor extended through the inferior vena cava and filled 2/3 of the right atrium. Echocardiography revealed normal cardiac function. Histopathologic findings after liver biopsy were consistent with hepatoblastoma. After 6 courses of chemotherapy including cisplatin and doxorubicin (PLADO, SIOPEL protocol), the cardiac tumor regressed completely. The patient's primary tumor was then fully resected; no cardiac surgery was performed. After surgery the AFP level was 4 IU/mL and echocardiography revealed normal cardiac function with no residual tumor. The patient has been in remission for 31 months postdiagnosis.