Management and outcomes of children with rhabdomyosarcoma in a low-to-middle-income country: A first report from Chris Hani Baragwanath Academic Hospital, South Africa

Abstract

BackgroundStudies done in South Africa show that Rhabdomyosarcomas (RMS) comprises 6 % of childhood malignancies. Very few centres in South Africa (SA) have reported their management and outcomes of children with RMS, and as such, it is difficult to compare outcomes and come up with management protocols befitting our environment. AimTo describe the management and outcomes of children with RMS at Chris Hani Baragwanath Academic Hospital (CHBAH). SettingThe Departments of Paediatric Surgery and Paediatric Oncology. MethodsA retrospective review of clinical records of patients below 18 years of age with RMS, managed from 01 January 2008 to 31 December 2017. ResultsFifty-eight patients had RMS, 77 % embryonal and 21 % alveolar subtypes. Primary tumour site was favourable in 48 %, and unfavourable in 45 %. Thirty-three patients (57%,) had surgery for the primary tumour, whilst 25 patients (43 %) did not have surgery. Post-operative clinical groups were 29 % group I, 9 % group II, 43 % group III, and 19 % group IV. The overall 5-year survival was 55%. Predictors for a good outcome included early disease stage at presentation, favourable site, embryonal subtype, and surgery for the primary tumour. ConclusionThe 5-year survival of 55 % from this study is low when compared to high income countries but is comparable to middle income countries. The main factor contributing to mortality is patients presenting with unresectable advanced disease in unfavourable sites. Surgical resection plays a major role in improving outcomes. Level of evidenceIV