Genitourinary rhabdomyosarcoma: lessons from a developing-world series

Abstract

ObjectiveThe objective was to retrospectively review a large series of pediatric patients with genitourinary rhabdomyosarcoma from a developing country. MethodsA total of 49 children were treated over a 47-year period (1961-2008). Analysis of the clinical presentation, demographics, surgical records, histological results, and oncological management was performed. The patients were analyzed as a whole and also in 2 separate groups (pre- and post-1992). ResultsThe median age at clinical presentation was 3½ years. The majority (59%) of patients were Intergroup Rhabdomyosarcoma Study group 3, with locally advanced disease at presentation. Twenty (41%) of the 49 patients presented with primary tumors greater than 10 cm in diameter. Sixteen (33%) of the 49 patients had positive regional lymph nodes at presentation. The overall survival of the series was 30 (65%) of 46. The survival for those treated after 1992 in Intergroup Rhabdomyosarcoma Study group 3 was superior (P = .04) to those treated before 1992 (80% vs 56%). ConclusionChildren in this large African series of genitourinary rhabdomyosarcoma present with greater locally advanced disease (node positive and bulky disease) when compared with the developed world. Improvements in the last 2 decades in local surgical and oncological care have led to an improvement in survival in children with locally advanced disease.