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Wilms' Tumour

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Shared care information

Please arrange for transfer of the patient after discussion with the paediatric oncologist on call.

New diagnosis of Wilms' Tumour

Please see Solid Tumours for information on work up for a new diagnosis of Wilms' Tumour

Involvement of Inferior Vena Cava by Wilms'

Renal vein involvement by Wilms' tumour is found in 11% of cases. Further extension into the IVC is seen in 4% and right atrial extension in ~0.7%.

It is very unusual for IVC/atrial involvement to be symptomatic but sometimes it produces tricuspid incompetence.

Vascular invasion of the renal vein, cava and atrium presents special surgical challenges, and, since these tumours will often respond to preoperative therapy, management requires careful consideration.

Tumour extension into the renal vein and proximate inferior vena cava can, in most cases, be removed en-bloc with the kidney. However, primary resection of tumours that extend up the inferior vena cava above the level of the hepatic veins and particularly to the atrium is associated with higher operative morbidity. In these circumstances, preoperative chemotherapy decreases the size and extent of the tumour thrombus without increasing its adherence to the vascular wall, thereby facilitating subsequent excision.

If after preoperative chemotherapy the tumour still extends above the hepatic veins, cardiopulmonary bypass is needed to remove the vascular extent of the tumour.

Investigations

  • Radiology
    • US with Doppler flow study of IVC
    • CT with IV contrast should be routinely performed on all cases of suspected Wilms' tumour
  • Biopsy of tumour if indicated. In some cases proceed to pre-operative chemotherapy with a clinical/radiological diagnosis of Wilms.

Management

  • Ensure the central venous catheter tip does not enter the right atrium
  • Commence chemotherapy as for unresectable disease according to current institutional protocol but omit actinomycin D if there is evidence of Budd-Chiari syndrome induced by tumour invasion. (Actinomycin D can cause sinusoidal obstruction syndrome which will be worsened in the setting of large vessel obstruction.)

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Document Control

  • Date last published: 24 February 2016
  • Document type: Clinical Guideline
  • Services responsible: National Child Cancer Network
  • Review frequency: 2 years