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Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery
1Department of Urology and Paediatric Oncology, University of Mainz, Germany. dragana.filipas@uro.akh.magwien.gv.at
Insights
Children with rhabdomyosarcoma (RMS) of the bladder and prostate treated with chemotherapy and surgery show excellent cure rates. Most patients achieved no evidence of disease, indicating successful treatment outcomes.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Medical Oncology
Background:
- Rhabdomyosarcoma (RMS) in pediatric patients affecting the bladder, prostate, or vagina requires effective treatment strategies.
- Long-term outcomes for this rare cancer are crucial for treatment optimization.
Purpose of the Study:
- To retrospectively analyze the treatment outcomes for children diagnosed with rhabdomyosarcoma (RMS) of the bladder, prostate, or vagina.
- To evaluate the effectiveness of chemotherapy, with or without radical surgery or radiotherapy, since 1968.
Main Methods:
- Retrospective analysis of 22 pediatric patients with RMS of the bladder/prostate or vagina treated between 1968 and 2001.
- Patients received primary polychemotherapy, primary surgery, or radiotherapy.
- Surgical interventions included radical cystoprostatectomy with various urinary diversions.
Main Results:
- After a mean follow-up of 8.6 years, 17 out of 22 patients had no evidence of disease.
- Five patients with advanced stages died from progressive RMS.
- Complications included ureteric reimplantation for stenosis and bladder augmentation for contraction post-radiotherapy.
Conclusions:
- Primary chemotherapy followed by radical surgery for RMS of the prostate and/or bladder enables complete tumor resection in most cases.
- This combined approach yields excellent cure rates for pediatric rhabdomyosarcoma.
Objective:
To retrospectively analyse the outcome of children with rhabdomyosarcoma (RMS) of the bladder, prostate or vagina who were treated with chemotherapy, with or without radical surgery or additional radiotherapy, at our institution since 1968.
Patients And Methods:
From a total of 107 children with RMS seen between 1968 and December 2001, 22 (mean age 5.9, range 0.5-18) had RMS of bladder/prostate or vagina. Twenty of the patients received primary polychemotherapy (vincristine, actinomycin D, cyclophosphamide, adriamycin, and more recently including etoposide and ifosfamide), two had primary surgery and seven had additional radiotherapy. Fourteen patients had radical cystoprostatectomy, with continent cutaneous urinary diversion with an ileocaecal pouch in seven, in one each a transverse colonic pouch, orthotopic ileocaecal bladder substitution, a rectal reservoir and rectosigmoid pouch and a colonic conduit diversion in two patients.
Results:
After a mean (range) follow-up of 8.6 (1.0-26) years, 17 patients had no evidence of disease. Five patients presenting initially with advanced tumour stages died from progressive RMS. Two patients with a continent urinary diversion required ureteric reimplantation for stenosis. In two patients severe bladder contraction after radiotherapy required bladder augmentation.
Conclusion:
Primary chemotherapy followed by radical surgery of RMS of the prostate and/or bladder allows complete tumour resection in most cases, and yields excellent cure rates.
