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

• Published in: BJU international 2000-08, Vol.86 (3), p.260-267
• Main Authors: El‐Sherbiny, M.T., El‐Mekresh, M.H., El‐Baz, M.A., Ghoneim, M.A.
• Format: Article
• Language: English
• Subjects: Adolescent; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; bladder salvage; chemotherapy; Child; Child, Preschool; children; Combined Modality Therapy; Disease-Free Survival; Diseases of the osteoarticular system; Female; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Medical sciences; Nephrology. Urinary tract diseases; Prostatic Neoplasms - mortality; Prostatic Neoplasms - therapy; Retrospective Studies; Rhabdomyosarcoma; Rhabdomyosarcoma - mortality; Rhabdomyosarcoma - therapy; Tomography, X-Ray Computed; Treatment Outcome; Tumors of striated muscle and skeleton; Tumors of the urinary system; Urinary Bladder Neoplasms - mortality; Urinary Bladder Neoplasms - therapy; Urinary tract. Prostate gland
• ISSN: 1464-4096; 1464-410X
• DOI: 10.1046/j.1464-410x.2000.00828.x

Objective 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. Patients and methods 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. Results 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. Conclusion 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.