Paediatric lower urinary tract rhabdomyosarcoma: a single-centre experience of 30 patients.

Abstract

To retrospectively analyse the long-term outcome of children with bladder and/or prostate rhabdomyosarcoma who were diagnosed at the authors' institution over the last 17 years. The study comprised 30 children (26 boys and four girls, mean age 5 years, range 15 days to 15 years); 23 had stage III and seven had stage II disease. The initial biopsy showed an embryonal variant in 27 and round-cell sarcoma in three patients. All patients received eight weekly doses of vincristine, actinomycin D and cyclophosphamide (VAC). Subsequent treatment depended upon the response to chemotherapy. Fourteen patients had a complete or partial response to chemotherapy (> 50% reduction in tumour size); they were maintained on VAC chemotherapy for 2 years. Twelve patients in this group survived with no evidence of disease for 7 months to 10 years. Additional therapies were used in three patients, i.e. radical cystectomy in one and external irradiation in two. Sixteen patients had a minimal response to chemotherapy; in six, radical cystectomy was feasible and was followed by one year of chemotherapy. All patients were free of disease for 4-11 years. Radiotherapy was given to the remaining 10 patients; thereafter radical cystectomy became feasible in five while partial cystectomy was possible in three. Only three of these 10 patients survived for 4-11 years. The tumour response to initial chemotherapy can be used to stratify patients into two risk-groups, i.e. low-risk patients with a complete or partial response in whom the bladder could be salvaged, and high-risk patients with a minimal response, in whom intensive treatment should be pursued, with no attempt at bladder salvage.