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Nephron-sparing surgery for bilateral Wilms′ tumours: A single-centre experience with 23 cases

Author(s): Millar AJW | Davidson A | Rode H | Numanoglu A | Hartley P | Desai Farieda

Journal: African Journal of Paediatric Surgery
ISSN 0189-6725

Volume: 8;
Issue: 1;
Start page: 49;
Date: 2011;
Original page

Keywords: Bilateral | nephron-sparing surgery Wilms′ tumour

Introduction: The challenge of management with bilateral Wilms′ tumours is the eradication of the neoplasm, while at the same time preserving renal function. Surgical management with a variety of nephron-sparing techniques, combined with chemotherapy and occasionally supplemented by transplantation has evolved over the last 30 years to achieve remarkable success. We document the experience of a single centre in a developing country. Material and Methods: Twenty-three bilateral Wilms′ tumours were seen in our service between 1981 and 2007. Treatment was, in most cases, according to National Wilms′ Tumour Study Group protocols, with initial bilateral biopsy, neoadjuvant chemotherapy, and tumourectomy. Technique of nephrectomy included full mobilization of the tumour-involved kidney, topical cooling with slush ice, vascular exclusion, tumour resection and reconstruction of the remnant kidney. Results: Twelve patients are alive and free of disease one to 15 years after treatment, all with well-preserved renal function (lowest glomerular filtration rate was 65 ml/min per (1.73 m 2 ). None of the survivors have hypertension. Eleven have died (two of unrelated disease) including six of the seven with spread outside the kidney. All three with unfavourable histology are alive. Four of the five metachronous presentations are alive, as are eight of 12 patients with synchronous bilateral tumours who presented since 2000. Conclusions: Appropriate chemotherapy and nephron-sparing surgery can achieve good results with preservation of adequate renal function in nearly all cases. Unfavourable histology did not have a reduced survival in our series. Metastatic spread outside the kidney had a poor prognosis.
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