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Background: Patients under age 4 with stage I favorable histology (FH) Wilms tumor have a reported survival advantage. Among children above 10 years, a poorer prognosis has been associated with a higher prevalence of diffuse anaplasia., Purpose: To determine if, in our practice, patients with Wilms tumors >8 years of age (stage II-V) have a poorer prognosis than those aged <8 years or <4 years., Procedure: Case-control study of 19 patients >8 years with Wilms tumor stages II-V who were identified from a cohort of 192 new patients (2002-2012). For each patient two controls were chosen matched for stage and histology, one 0-3 years and one 4-7 years. Neo-adjuvant chemotherapy was offered to all, combined with intensive supportive care. Postoperative treatment was determined by local stage and histology. OS and EFS at 5 years for the different age groups were compared., Results: Each age group contained 19 patients, of whom 6 had stage II tumors, 3 stage III, 8 stage IV, and 2 stage V. Histology was intermediate risk (IR) in 17 and high risk (HR) in 2. OS at 5 years was 80.8% and EFS was 79.2% for the whole group. No significant difference in outcome could be shown between age groups. Loss to follow up was 6/57 (11%)., Conclusions: The survival advantage of young age (<4 years) associated with stage I FH could not be demonstrated in higher stages. Age had no significant impact on prognosis although a trend to better outcome was seen in children <4 years., (© 2014 Wiley Periodicals, Inc.)

Background: Liver resections are widely performed in paediatric surgery. Many techniques exist to achieve vascular control, minimise bleeding and complete the parenchymal division., Methods: We retrospectively reviewed all liver resections performed in the Department of Paediatric Surgery at our institution between January 2005 and June 2012. Data pertaining to basic demographics, indications for surgery, parenchymal transection techniques, morbidity, mortality and histology were collated., Results: Twenty-one resections were performed in children aged 6 weeks - 11 years; 18 for malignant liver disease (including 9 hepatoblastomas), and 3 for benign disease. We describe 1 peri-operative mortality secondary to torsion of the liver remnant, and no surgical morbidity. Three cases underwent total hepatic vascular exclusion with sharp parenchymal transection. The remaining patients underwent selective vascular inflow and outflow control using the Cavitron Ultra Sonic Aspirator and Harmonic Scalpel to divide the parenchyma., Conclusion: Care for these patients should be multidisciplinary. High-volume units and access to liver transplantation offer optimal results. No technique is proven superior to the 'clamp crush' technique of parenchymal transection. Knowledge of hepatic anatomy is key to minimising morbidity, and surgeons should be familiar with and have the flexibility to use all techniques of vascular control.

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.

Background: In Africa, Wilms tumour frequently presents with advanced disease. This study reports our results over 25 years using the National Wilms Tumour Study Group (NWTSG) approach of primary surgery followed by chemotherapy., Procedure: A retrospective analysis was performed on all patients diagnosed with unilateral Wilms tumour between January 1979 and December 2003., Results: The records of 188 children with newly diagnosed Wilms tumour were examined. Of these 57 (30.3%) were stage I, 33 (17.6%) were stage II, 60 (31.9%) were stage III and 38 (20.2%) were stage IV. Twenty-four patients (12.8%) had unfavourable histology (UFH). The estimated 5-year overall survival (OS) for the whole group was 80.5% (84.8% for favourable histology (FH) and 51.6% for UFH). Among those with FH, estimated 5-year OS was 94.4% for stage I, 96.2% for stage II, 84.9% for stage III and 54.2% for stage IV. There was no difference in OS between those FH stage III tumours that were operable and those deemed inoperable. Intra-operative spillage was uncommon (8%), and did not increase local relapse rate., Conclusions: National Wilms Tumour Study Group protocols employed in an African setting with highly competent and experienced surgical care, produced results for non-metastatic FH Wilms tumour comparable to those of the NWTSG.