SURGICAL-MANAGEMENT OF WILMS-TUMOR WITH INTRACARDIAC NEOPLASTIC EXTENSION

In the management of Wilms tumor (nephroblastoma) with intracardiac ex tension (ICE) an interdisciplinary approach is mandatory. The three ca ses reported here reflect the impressive improvements of both diagnost ic facilities and surgical strategies over the last 16 years as eviden ced 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 cardiopulmon ary bypass (CPB) and deep hypothermic circulatory arest (DHCA). In the first child (1976) only tumor nephrectomy was performed. Under chemot herapy fatal massive pulmonary embolism occurred. In the second child (1980) ICE was removed after tumor nephrectomy on an emergency basis u sing CPB. The third child (1987) was operated on electively using the concepts of CPB + DHCA. Now, both children are doing well without evid ence of disease 13 years (case 2: stage III) and 6 years (case 3: stag e IV after an aggressive adjuvant-therapy regimen of multiagent chemot herapy and radiation. In conclusion, in children with nephroblastoma a nd ICE an aggressive surgical approach and subsequent multiagent chemo therapy are advocated.