Surgery of cavoatrial tumor thrombus in nephroblastoma: a report of the SIOP/GPOH study

Pediatr Blood Cancer. 2004 Jul;43(1):40-5. doi: 10.1002/pbc.20056.


Background: Resection 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.

Procedure: In 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.

Results: The 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.

Conclusion: Our 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.

MeSH terms

  • Cardiopulmonary Bypass*
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Female
  • Germany / epidemiology
  • Heart Atria* / pathology
  • Heart Atria* / surgery
  • Humans
  • Hypothermia, Induced
  • Infant
  • Intraoperative Complications / epidemiology
  • Kidney Neoplasms / mortality
  • Kidney Neoplasms / pathology
  • Kidney Neoplasms / surgery*
  • Male
  • Neoplasm Invasiveness
  • Neoplasm Recurrence, Local / epidemiology
  • Retrospective Studies
  • Survival Rate
  • Thrombosis / mortality
  • Thrombosis / surgery*
  • Vena Cava, Inferior* / pathology
  • Vena Cava, Inferior* / surgery
  • Wilms Tumor / mortality
  • Wilms Tumor / pathology
  • Wilms Tumor / surgery*