Radical pelvic surgery has no role in primary management of vesical or prostatic rhabdomyosarcoma

BMJ 1996; 313 doi: https://doi.org/10.1136/bmj.313.7055.492b (Published 24 August 1996) Cite this as: BMJ 1996;313:492
  1. G Humphrey, Senior registrar in paediatric surgery,
  2. B R Squire, Consultant paediatric surgeon
  1. Children's Day Hospital, St James's University Hospital, Leeds LS9 7TF

    EDITOR,—Chris Dawson and Hugh Whitfield's article on common paediatric problems in urology fails adequately to reflect advances in the management of rhabdomyosarcomas of the urinary tract in childhood and may result in inappropriate management of children with this difficult malignancy.1 The role of surgery in the management of this tumour has evolved over the past 30 years in an attempt to balance long term survival with morbidity secondary to urinary tract surgery and radiotherapy.2

    Before 1960 the treatment of rhabdomyosarcoma of the urinary tract consisted of radical surgery and radiotherapy alone or in combination; there were few long term survivors. By the late 1970s wide surgical excision had been shown to be curative in 73% of localised tumours, but the prognosis for advanced disease remained poor.2 Pelvic rhabdomyosarcomas were among the first localised sarcomas to be treated by primary chemotherapy in an attempt to maintain continuity of the urinary tract. Protocols I-III of the international rhabdomyosarcoma study have assessed the role of chemotherapy and surgery in the management of rhabdomyosarcoma of the urinary tract, with protocol III recently showing that primary chemotherapy in non-metastatic tumours permits preservation of the bladder in 60% without compromising long term survival rates of about approximately 75%.2 3

    On the basis of the results of protocols I-III,2 3 and in contrast to the view expressed by Dawson and Whitfield, paediatric surgeons and oncologists believe that radical pelvic surgery has no role in the primary management of vesical or prostatic rhabdomyosarcoma, although it offers the prospect of cure in children who do not have disseminated disease but fail to respond to chemotherapy or develop a pelvic relapse. Limited excisional surgery retains a role in the primary management of tumours confined to the anterior wall or dome of the bladder.4

    The role of bladder preserving surgery in prostatic rhabdomyosarcoma (that is, radical prostatectomy) remains unclear because of the small numbers of children who have been managed this way; such surgery is currently reserved as an alternative to anterior pelvic exenteration when the primary tumour has failed to respond to prolonged chemotherapy or has relapsed locally.3

    Dawson and Whitfield should have said that the combination of chemotherapy, radiotherapy, and limited surgery reduces the need for radical surgery in patients with genitourinary rhabdomyosarcoma.


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