Nephron-sparing surgery for bilateral Wilms' tumours: a single-centre experience with 23 cases.

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.

Carboplatin-based chemotherapy and surgery: a cost effective treatment strategy for malignant extracranial germ cell tumours in the developing world.

Nineteen children with malignant extracranial germ cell tumours (MEGCT), were treated at our institution with a combination of carboplatin-based chemotherapy and surgery from 1992 to 2008. Seventeen are long term survivors. Chemotherapy was administered on an outpatient basis and required no monitoring of GFR or auditory function. There were no deaths from toxicity or infection. Blood product use was low. Carboplatin-based therapy achieved excellent results in our patient population and constitutes more than a US $1,800 saving per patient compared to regimens containing cisplatin. This has application in resource limited settings.

The evolving management of Burkitt's lymphoma at Red Cross Children's Hospital.

BACKGROUND: Treatment for Burkitt's lymphoma at Red Cross Children's Hospital has evolved from the use of aggressive surgery and less intensive chemotherapy to a conservative surgical approach with more intensive chemotherapy. METHODS: The study was a retrospective folder review of patients diagnosed with Burkitt's lymphoma at RCCH between 1984 and 2004. RESULTS: Ninety-two children were treated for Burkitt's lymphoma at RCCH between 1984 and 2004. There were 10 patients with group A or fully resected disease, 52 with group B or extensive localised disease, and 30 with dissemination to the bone marrow and/or central nervous system or group C disease. Protocol 1 (less intensive chemotherapy based on the COMP regimen) was used from 1984, with protocol 2 (more intensive chemotherapy based on the LMB regimen) introduced in 1988 for group C disease, 1991 for group B disease and 1996 for group A disease. Overall 5-year survival increased from 20% with protocol 1 to 66% with protocol 2 for group C disease, and from 76.5% with protocol 1 to 88.2% with protocol 2 for group B disease. There were more admissions for neutropenic fever in patients on protocol 2 and more episodes of mucositis, and these patients required more red cell and platelet transfusions. With a more conservative surgical approach, biopsy largely replaced attempts to partially resect the tumour at primary surgery, and there was a consequent decline in surgical complications. CONCLUSIONS: Intensive chemotherapy with protocol 2 has resulted in improved survival for group C and group B patients, but with more morbidity. Protocol 1, which is less intensive with less morbidity, remains a viable strategy for group A and group B disease in resource-poor settings.

Intraocular rhabdomyosarcoma in the sibling of a patient with a cerebellar medulloepithelioma.

A 12-year-old girl with a three-year history of right eye disease was referred to our service after enucleation of her right eye. Histology showed a pleomorphic high-grade rhabdomyosarcoma. As there was extrascleral extension, we elected to treat her with local radiotherapy and adjuvant vincristine, dactinomycin, cyclophosphamide, and adriamycin. She remains disease free 35 months from diagnosis. Notably, a younger sibling died in early childhood as a result of a cerebellar medulloepithelioma. Although there were no teratoid elements in the rhabdomyosarcoma, our patient's unusual disease course would be consistent with rhabdomyosarcomatous differentiation in a teratoid medulloepithelioma.

Wilms tumour experience in a South African centre.

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.

Bilateral Wilms' tumors: a single-center experience with 19 cases.

BACKGROUND/PURPOSE: Bilateral Wilms' tumors present a therapeutic challenge, particularly with delay in presentation, when there is poor response to chemotherapy and when associated with nephroblastomatosis. The primary aim of management is eradication of neoplasm, while at the same time preserving of renal function. METHODS: Nineteen bilateral Wilms' tumors were seen in our service between 1981 and 2003. This represented 10% of the 190 patients with Wilms' tumors seen during this period. There were 12 female and 7 male patients ranging in age from 7 months to 8 years. Fourteen had synchronous presentation, one of whom had liver metastasis at diagnosis. Of the 5 patients with metachronous tumors, 3 had their initial nephrectomies done elsewhere. Nephroblastomatosis was identified in 18 (95%) of the patients. Treatment was, in most cases, according to National Wilms Tumor Study Group protocols, with initial bilateral biopsy, neoadjuvant chemotherapy, and tumorectomy. Where indicated, nephrectomy (partial or complete) involved using ice dam topical cooling and vascular control, and in one case, bench surgery and extensive renal reconstruction with orthotopic autotransplantation. Revision tumorectomy was used on 3 occasions for recurrence in areas of nephroblastomatosis. There were 6 extrarenal relapses. RESULTS: Ten patients are alive and free of disease 1 to 15 years after treatment, all with well preserved renal function (lowest recorded glomerular filtration rate was 85 mL/min per 1.73 m2). Nine have died (2 of unrelated disease), including 6 of the 7 with spread outside the kidney. All 3 with unfavorable histology are alive. Of the 5 with metachronous presentations, 4 are alive, as are 7 of 10 who presented in the last decade. CONCLUSIONS: Appropriate chemotherapy and conservative nephron-sparing and innovative surgery can achieve good results with preservation of adequate renal function in nearly all cases. Nephroblastomatosis was an almost universal finding and requires close monitoring because Wilms' tumors developed in residual suspect areas. Revision surgery was effective. Unfavorable histology did not have a reduced survival in our series. Metastatic spread outside the kidney had a poor prognosis.