• Philipp Szavay, Tobias Luithle, Oliver Semler, Norbert Graf, and Joerg Fuchs.
• Department of Pediatric Surgery, University of Tuebingen, Tuebingen, Germany. Philipp.Szavay@med.uni-tuebingen.de
• Pediatr Blood Cancer. 2004 Jul 1;43(1):40-5.

BackgroundResection of a Wilms tumor extending through the inferior vena cava into the right atrium represents a challenge to the pediatric surgeon. Exact preoperative diagnosis is essential to identify the tumor and its intravascular extension. To achieve a complete excision of the tumor cardiopulmonary bypass and hypothermia may be required. The feasibility of a complete resection is important as it guides subsequent therapy such as chemotherapy and radiation.ProcedureIn order to define these issues, we reviewed the records of 33 of 1,151. Patients enrolled in the SIOP 93-01/GPOH Study and the SIOP 2001/GPOH Study who had a tumor thrombus into the inferior vena cava and into the right atrium.ResultsThe median age at diagnosis was 3.73 years. Twenty-four patients had a tumor thrombus into the inferior vena cava, in nine patients the thrombus reached into the right atrium. All patients were operated on; cardiopulmonary bypass was used in nine patients. There were no deaths intraoperatively. Twenty-nine children are still alive; four patients died, one patient due to aspiration and failed resuscitation, two patients died from a recurrent tumor, and one child due to an unresectable primary tumor.ConclusionOur report suggests that Wilms tumor extending to the inferior vena cava and the right atrium is technical challenging, but with adequate preoperative diagnosis and a multidisciplinary surgical approach including cardiopulmonary bypass and hypothermia, the prognosis is favorable.Copyright 2004 Wiley-Liss, Inc.

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