Surgical management of Wilms tumor with intracardiac neoplastic extension

In the management of Wilms tumor (nephroblastoma) with intracardiac extension (ICE) an interdisciplinary approach is mandatory. The three cases reported here reflect the impressive improvements of both diagnostic facilities and surgical strategies over the last 16 years as evidenced in the literature. Nowadays, prerequisite for a well-planned, safe, and successful operation is the exact delineation of the intravasal tumor extension and the use of the combined techniques of cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). In the first child (1976) only tumor nephrectomy was performed. Under chemotherapy fatal massive pulmonary embolism occurred. In the second child (1980) ICE was removed after tumor nephrectomy on an emergency basis using CPB. The third child (1987) was operated on electively using the concepts of CPB+DHCA. Now, both children are doing well without evidence of disease 13 years (case 2: stage III) and 6 years (case 3: stage IV) after an aggressive adjuvant-therapy regimen of multiagent chemotherapy and radiation. In conclusion, in children with nephroblastoma and ICE an aggressive surgical approach and subsequent multiagent chemotherapy are advocated.