Wilms' tumor with intracardiac extension: chemotherapy before surgery

The article describes two Chinese boys ages 2 and 3 years with unilateral Wilms' tumors complicated by intracaval and intracardiac extension. In contrast to the previously recommended treatment with surgery followed by chemotherapy and radiation therapy, the children were managed primarily with combination chemotherapy before definitive operation. Reduction of tumor size on serial imaging was documented, and no viable tumor cells were found when the involved kidney and right atrium were explored. Both patients remained alive without evidence of disease more than 5 years after initial diagnosis. A literature search revealed case reports and retrospective analyses of 70 patients with Wilms' tumors and intracardiac involvement, and a tendency toward preoperative chemotherapy with or without the addition of radiation therapy was observed. The overall outcome of this group of patients parallels the outcome of those without intracardiac extension by histology and stage. Wilms' tumor presenting with extension into the inferior vena cava and right atrium is thus rare and renders the affected child with additional cardiovascular complications and operative risks. As a result of the uncommon occurrence, a consensus on management based on prospective study would be difficult. The present report and the literature are supportive of the use of preoperative chemotherapy in the initial management of advanced Wilms' tumor extending into the right atrium.