title: Management of nine cases of Wilms' tumour with intracardiac extension - a single centre experience.
abstract: OBJECTIVE
  The aim of this study was to review the management of children with Wilms' tumour who have intracardiac extension.
  
  
  METHODS
  Data were collected from patient notes regarding presentation, operative details, and outcome.
  
  
  RESULTS
  From 1984 through 2011, 264 children with Wilms' tumour were treated at our hospital. Nine (3.4%) had cavo-atrial extension of the tumour thrombus. The thrombus extended into the right ventricle in two children and involved the hepatic veins in both, and also two others. Pre-operative chemotherapy was administered in eight children with complete regression of the intra-cardiac tumour thrombus in two cases. One child died preoperatively of septicaemia and respiratory failure after two doses of chemotherapy. Six children with intra-cardiac tumour were operated on under cardiopulmonary bypass (CPB) with deep hypothermia and circulatory arrest (DHCA). The mean ischemic time was 30 min. There was one peri-operative death in a child with hepatic vein involvement and Budd-Chiari syndrome. All others made a good postoperative recovery. All tumours were favourable histology. To date four children are still alive and disease free. Three children have died as a result of pulmonary metastases.
  
  
  CONCLUSIONS
  Intracardiac extension of Wilms' tumour is rare, and the management is technically challenging. Pre-operative chemotherapy is effective. CPB and DHCA for excision of the cavo-atrial tumour thrombus may be necessary. Distant metastatic disease is common and determines long term prognosis. Hepatic vein extension complicates surgery and remains challenging.
authors: School of Child and Adolescent Health, Divisions of Paediatric Surgery, Cardiothoracic Surgery and Haematology/Oncology, Red Cross War Memorial Children's Hospital, Rondebosch, 7700 Cape Town, South Africa.
authors: Yusuf
authors: Y
authors: Abdullah
authors: Jonathan
authors: J
authors: Karpelowsky
authors: Alan
authors: A
authors: Davidson
authors: Jenny
authors: J
authors: Thomas
authors: Andre
authors: A
authors: Brooks
authors: John
authors: J
authors: Hewitson
authors: Alp
authors: A
authors: Numanoglu
authors: Sharon
authors: S
authors: Cox
authors: Alastair J W
authors: AJ
authors: Millar
mesh_terms: D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D006338:Heart Neoplasms / Q000556:secondary* / Q000628:therapy*; D006801:Humans; D007680:Kidney Neoplasms / Q000473:pathology*; D008297:Male; D009360:Neoplastic Cells, Circulating; D009396:Wilms Tumor / Q000556:secondary* / Q000628:therapy*