Wilms' tumour with persistent intravascular extension: A review of the surgical aspects of management

Murthi, G.V.; Kocyildirim, E.; Sellathury, S.; Cuckow, P.M.; Wilcox, D.T.; Michalski, A.; Sebire, N.J.; Elliott, M.J.; Duffy, P.G.

doi:10.1016/j.jpurol.2005.10.004

« Previous Next »Journal of Pediatric Urology
Volume 2, Issue 5 , Pages 439-445, October 2006

Wilms' tumour with persistent intravascular extension: A review of the surgical aspects of management

G.V. Murthi
- Department of Paediatric Urology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK
- Corresponding author. Tel.: +44 116 2718814; fax: +44 115 9709006.
E. Kocyildirim
- Department of Cardio-Thoracic Surgery, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK
S. Sellathury
- Department of Paediatric Urology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK
P.M. Cuckow
- Department of Paediatric Urology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK
D.T. Wilcox
- Department of Paediatric Urology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK
A. Michalski
- Department of Haemato-Oncology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK
N.J. Sebire
- Department of Pathology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK
M.J. Elliott
- Department of Cardio-Thoracic Surgery, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK
P.G. Duffy
- Department of Paediatric Urology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK

Received 12 July 2005; accepted 4 October 2005.

Abstract

Aim

To review the surgical management of Wilms' tumour with persistent intravascular (vena caval +/− atrial) tumour extension.

Patients and methods

Data were collected regarding operative details, tumour and ‘thrombus’ histology, and long-term outcome for patients with Wilms' tumour with cavo-artial extension.

Result

From 1988 to 2004, 13 patients underwent treatment for Wilms' tumour with persistent intravascular extension. Preoperative chemotherapy was administered in 11/13 patients and postoperative radiotherapy in eight patients. Intravascular involvement was upto IVC (5), and right atrium (8) patients. Techniques employed for excision of intra-vascular component were: local cavotomy (3), extensive infra-diaphragmatic cavotomy without cardiopulmonary bypass (CPB) (1), and excision of cavo-atrial tumour with CPB (+/− hypothermia and cardiac arrest) (9). Mean time on CPB was 90 min. Caval repair was accomplished by primary repair (6) and pericardial graft in (7) patients. There were no intraoperative deaths and few major complications.

Tumour thrombus contained malignant cells in 10/13 cases. Mean follow up has been for 55.4 months. To date, seven patients remain disease-free (one lost to follow up), disease recurred in five patients, three of whom have died. There were no symptoms related to the graft.

Conclusions

Surgery for Wilms' tumour with persistent intravascular extension despite chemotherapy is technically challenging. CPB +/− hypothermia and cardiac arrest and extensive caval repair with a graft is safe and reliable in the long term.

Keywords: Wilms' tumour, Surgery, Persistent Intravascular extension, Cardiopulmonary bypass