Journal of pediatric surgery · 2013
Management of nine cases of Wilms' tumour with intracardiac extension - a single centre experience.
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Abdullah Yusuf, Karpelowsky Jonathan, Davidson Alan, Thomas Jenny, Brooks Andre, Hewitson John, Numanoglu Alp, Cox Sharon, Millar Alastair J W
Аннотация
AIM: The aim of this study was to review the management of children with Wilms' tumour who have intracardiac extension.
PATIENTS AND 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.
CONCLUSION: 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.
PATIENTS AND 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.
CONCLUSION: 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.