Wilms’ tumour in African children: Can an institutional approach improve outcome?

Background:

The poor outcome for patients with Wilms’ tumour (WT) in developing countries has been predicated on late presentation, poverty and low rate of chemotherapeutic access. This study aims to evaluate the effects of an institutionalised approach to improving outcome for patients managed in a tertiary hospital in Nigeria.

Materials and Methods:

Oncology records of children diagnosed with WT between 2009 and 2013 were analysed for therapy completion and other prognostic parameters. Ensuing data were then compared with those from other centres in Africa.

Results:

Compared with results from some local and African studies, the therapy completion rate was higher (60%) with a survival rate among this group being between 1 and 4 years. No patient was lost to follow-up because of unavailability or unaffordability of cytotoxic agents.

Conclusion:

This study shows that an institutionalised approach can help to improve access to anti-cancer drugs, reduce the rate of loss to follow-up and thus improve outcome. There is however need to improve on patient-doctor communication, form support groups and establish a WT registry.     

Spinal cord compression in children with Wilms’ tumour

Purpose  

To document the clinical, imaging and histopathological features of five children with paraplegia due to Wilms’ tumour (WT), highlighting therapeutic options and patient outcome in a developing country.     

Bilateral Wilms’ tumour in a developing country: a descriptive study

Purpose

To present our experience of 20 children with bilateral Wilms’ tumour seen in a resource-challenged environment over a 10-year period.

Method

All patients with a diagnosis of bilateral synchronous Wilms’ tumour were identified and recruited.

Results

Study patients represented 11 % of a cohort of 177 new patients with Wilms’ tumour seen over the same period. Three patients had a syndromic predisposition to Wilms’ tumour. Metastatic disease was seen at presentation in four patients (20 %) and three children presented with unilateral tumour rupture. One patient presented with paraplegia and one with obstruction of the duodenum. All children received neoadjuvant chemotherapy. One HIV-infected child died of IRIS after neoadjuvant treatment, but before surgery. One child died of progressive disease after unilateral nephrectomy. Nephron-sparing surgery was performed in 22 kidneys and 15 kidneys were removed in toto. Following enucleation of tumours, three children had positive margins. Discordant histopathology was seen in 53 % of patients. Overall survival at 2 years is 85 %.

Conclusion

Despite significant co-morbidity and advanced disease, bilateral Wilms’ tumour is a treatable disease in a resource-constrained environment.     

Emergent embolisation to control severe haematuria in Wilms’ tumour

Wilms tumour commonly presents with an abdominal mass and gross haematuria. Here, we present the novel application of paediatric renal arterial embolisation to control life-threatening haematuria in Wilms tumour.     

Metastatic renal abscess mimicking Wilms’ tumour

Renal abscesses are uncommon in children, and usually present with a high fever and flank pain. We report a 9-year-old girl who presented with abdominal pain and cough. While the finding of multiple lung nodules and a renal mass prompted the initial diagnosis of Wilms tumour, biopsy showed multiple renal abscesses with secondary lung involvement. The imaging findings and pathology correlation are provided.     

The morbidity and outcome of surgery in children with large pre-treated Wilms’ tumour: size matters

Sixty-seven patients with resected Wilms’ tumour > 1.0 kg are reported. Surgery in this group is difficult, may result in the sacrifice of adjacent structures, and frequently upstages the patient. Mortality is related to tumour burden. Whilst there is little agreement on the therapeutic implications of failure to respond to neoadjuvant therapy, these surgical and anaesthetic hazards must be recognized, and alternative stratagems considered, prior to attempting a surgical procedure.     

Preoperative angioembolisation for life-threatening haemorrhage from Wilms’ tumour: a case report

Wilms tumour is one of the most common abdominal tumours of childhood. Severe perirenal bleeding resulting in consumptive coagulopathy and colonic obstruction are rare complications of Wilms tumour. We present a case report of one patient with these two complications, their successful management with preoperative angioembolisation and emergency nephrectomy, and a review of the relevant literature.     

Botryoid Wilms’ tumor: report of two cases

Background  

Botryoid Wilms’ tumor is a rare kind of Wilms’ tumor. We report two cases of this tumor.     

Efficacy of Preoperative Chemotherapy in Treatment of Children With Wilms’ Tumor: A Meta-Analysis

Context:

To assess the efficacy of preoperative chemotherapy in Wilms’ tumor patients and explore its true value for specific subgroups.

Objectives:

In the presence of these controversies, a meta-analysis that examines the efficacy of preoperative chemotherapy in Wilms’ tumor patients and specific subgroups is needed to clarify these issues. The objective of this meta-analysis is to assess the efficacy of preoperative chemotherapy in Wilms’ tumor patients and explore its true value for specific subgroups.

Data Sources:

Computer-based systematic search with “preoperative chemotherapy”, “Neoadjuvant Therapy” and “Wilms’ tumor” as search terms till January 2013 was performed.

Study Selection:

No language restrictions were applied. Searches were limited to randomized clinical trials (RCTs) or retrospective studies in human participants under 18 years. A manual examination of references in selected articles was also performed.

Data Extraction:

Relative Risk (RR) and their 95% Confidence Interval (CI) for Tumor Shrinkage (TS), total Tumor Resection (TR), Event-Free Survival (EFS) and details of subgroup analysis were extracted. Meta-analysis was carried out with the help of the software STATA 11.0. Finally, four original Randomized Clinical Trials (RCTs) and 28 retrospective studies with 2375 patients were included.

Results:

For preoperative chemotherapy vs. up-front surgery (PC vs. SU) group, the pooled RR was 9.109 for TS (95% CI: 5.109 - 16.241; P < 0.001), 1.291 for TR (95% CI: 1.124 - 1.483; P < 0.001) and 1.101 for EFS (95% CI: 0.980 - 1.238; P = 0.106). For subgroup short course vs. long course (SC vs. LC), the pooled RR was 1.097 for TS (95% CI: 0.784 - 1.563; P = 0.587), 1.197 for TR (95% CI: 0.960 - 1.493; P = 0.110) and 1.006 for EFS (95% CI: 0.910 - 1.250; P = 0.430).

Conclusions:

Short course preoperative chemotherapy is as effective as long course and preoperative chemotherapy only benefits Wilms’ tumor patients in tumor shrinkage and resection but not event-free survival.     

Wilms’ Tumor- Roadmaps of Management

The management of Wilms’ tumor emerging of the outstanding clinical trials undertaken in the developed world in the last four decades has led to excellent longterm outcomes. The scenario in developing country like India is different; late presentation with massive tumors and advanced stage, lacunae in staging, nonavailability of pediatric medical oncologists and poor follow-ups are common. A comprehensive summary of available therapeutic modalities is provided here along with clear roadmaps of management of Wilms’ tumor as per Société Internationale d’Oncologie Pédiatrique (SIOP) and National Wilms’ Tumor Study Group/Children’s Oncology Group (NWTSG/COG) protocols in simple tabulated form.     

Clinicopathological profile of Wilms’ tumor

The profile of renal tumors in children less than 15 years of age during the period 1991–1997 is presented. Among the 37 children with kidney tumors, 29 (78.4%) had Wilms′ tumor. There was also a 20-year-old female with Wilms′ tumor. The median age at presentation was 2.6 years (range 2.5 months to 20 years). 66.7% of the cases diagnosed were ≤3 years and 90% were ≤6 years. Five cases were under one year of age. The male to female ratio was 2 : 1. Twenty-two cases (73.3%) were triphasic and 7 (23.3%) were biphasic. Only one case was monophasic with blastemal component. Five cases (16.7%) showed nephrogenic rests in the uninvolved renal parenchyma and one case had nephroblastomatosis. The tumor was favorable in 26 cases (86.7%) and unfavorable in 4. Fourteen cases were in-patients while 16 were outside referrals. The pathological (10 cases whose specimens were sent from other centers) and clinicopathological (13 hospitalized patients) staging showed 10 cases (43.5%) with stage 1,4 cases (17.4%) with stage 2, and 7 cases (30.4%) with stage 3. In two cases (8.7%), there was stage 4 disease. The length of the follow-up period in the 13 hospitalized patients ranged from 7 days to 5 years 5 months (median 14 months). There was one recurrence and one death after 2 years of diagnosis.     

A surgical approach to Wilms’ tumour with retrohepatic vena caval extension

Purpose

Wilms’ tumours (WT) with retrohepatic vascular extension traditionally requires cardiac bypass for complete excision. We share our experience of these complex cases.

Methods

A retrospective review was performed of children with WT with retrohepatic vascular extension presenting to two UK children’s hospitals. Tumour stage, chemotherapy, level of vascular extension, operative details and complication data were analysed.

Results

Ten children were identified. Mean age 6.6 years (range 3.3–8.2 years); tumour side 6 right, 2 left, 2 bilateral. Level of tumour extension was to the right atrium in two, diaphragm in two, hepatic vein (HV) level in four and retrohepatic inferior vena cava (IVC) in one patient. Following chemotherapy it reduced to hepatic veins (5) or below (4). Surgery involved radical nephrectomy and complete mobilisation of the liver off the IVC, which was then clamped, opened and the thrombus excised. There were no intraoperative complications. Mean hospital stay was 9.77 days (7–20 days). Histology showed viable tumour thrombus in six patients. One patient died after 1 year from metastatic disease.

Conclusion

Retrohepatic extension of WT can be managed without bypass using pre-operative chemotherapy and by complete liver mobilisation. The tumour was always adherent to IVC and required sharp dissection.     

Botryoid Wilms’ tumor: a report of two cases

We report two rare cases of botryoid Wilms tumor (BWT) occupying the renal collecting system with no macroscopic parenchymal mass. In case 1, a 3-year-old boy presented with a mass in the right flank, low-grade fever, abdominal pain, and macrohematuria. Radiology revealed an enlarged right kidney with a heterogeneous mass occupying a large part of the dilated renal calyx, pelvis, and ureter. Radical right nephroureterectomy was performed. The histopathologic diagnosis was nephroblastoma, and the pedicle of the tumor was attached to the renal parenchyma near the pelvic wall. In case 2, a 2-year-old boy presented with macrohematuria, and the clinical course was almost the same as in case 1. No local recurrence or metastatic spread has been detected for 4 years postoperatively in case 1 and for 9 months postoperatively in case 2.     

Is Pre-operative Chemotherapy Desirable in all Patients of Wilms’ Tumor?

The timing and role of chemotherapy in the management of Wilms’ tumor has long been the matter of debate, with different groups showing equally comparable and encouraging results. Over the last decade, however, both the ideol-ogies seem to be converging and the attempt has been to identify groups benefitting with pre-operative chemotherapy, as well as those, where upfront resection should be attempted. In this article authors intend to discuss pros and cons of both the strategies and their applicability in a resource poor setting in developing countries like India.     

Management and prognosis of adrenocortical tumors in children: can we find out an appropriate points-scoring system to predict prognosis?

Background/purpose

Adrenocortical tumors (ACTs) are rare in childhood. There are no recognized criteria to exactly distinguish between benign and malignant forms, or predict prognosis. The incidence of tumor varies across geographic regions or ethnicities, as well as malignant proportion. The aim of this study is to examine a single institution’s experience with pediatric ACTs and to validate the prognostic value of the biologic/pathologic criteria of Wienecke.

Methods

Records of 26 pediatric ACTs between 1994 and 2016 in our center were reviewed retrospectively. The data recorded of each patient included clinical characteristics, treatment, pathologic findings, disease stating, and outcome. Tumors were categorized according to the Wienecke criteria.

Results

All patients underwent primary surgical excision, including negative margins in 20 cases. Stage distribution at diagnosis was: ST I 12, ST II 8, ST III 5, and ST IV 1. According to Wienecke scoring system, 13 cases were <3 criteria, 6 cases were =3 criteria, and 7 cases were >3 criteria. At median follow-up of 34.5 months, 18 patients survived without evidence of disease and 8 patients had lethal outcome. There was a strong association between high Wienecke score and both high stage and adverse outcome.

Conclusions

Wienecke criteria can be an appropriate points-scoring system to predict prognosis for adrenocortical tumors in children. Complete surgical resection with negative margins is optimal for survival.