Experience and outcomes of nephroblastoma in Johannesburg, 1998-2003

Introduction: Outcomes for children with cancer in the developing world are compromised by the difficulties for patients in accessing health services and by competition for resources between oncological services and the myriad other health problems of emerging nations. The purpose of this study is to document and analyse our experience and the outcomes of children with nephroblastoma over recent years. Methods: This is a retrospective review of all patients who underwent combined oncological and surgical treatment for nephroblastoma in the Paediatric Oncology Unit between 1998 and 2003. Results: Sixty-three patients were treated for Wilms' tumour; the mean age was 3 years 8 months (range 4 months to 11 years). The majority of children presented with an abdominal swelling or mass. Preoperative chemotherapy was given in forty-six cases (73%). The tumour stage distribution was 11/63 stage I (17%), 11/63 stage (17%), 21/63 stage III (33%),16/63 stage IV (25%) and 4/63 stage V (6%). Postoperative chemotherapy and radiotherapy was given according to the SIOP protocol. During the study period, thirteen patients (21%) died (7 cancer-specific, 2 postoperative, 4 sepsis related), thirteen (21%) were lost to follow-up and thirty-seven (59%) are free of disease with a mean follow-up period of 3.67 years. Children with stage I and stage II had a disease-free survival at 4 years of 89%. However, those with stage III, IV and V disease had 4-year survival of 66.75% (p = 0.07). Overall, four-year post-nephrectomy survival was 76%. Conclusion: Outcomes for children with cancer have improved dramatically over recent years; however, in the developing world, the scarcity of hospital resources and the overwhelming burden of non-cancer diseases can mean that oncological treatment is extremely challenging. In our society, children tend to present with nephroblastoma at an advanced stage; however, treatment by dedicated, multidisciplinary teams can achieve good results.