title: Children with vesical rhabdomyosarcoma (RMS) treated by partial cystectomy with neoadjuvant or adjuvant chemotherapy, with or without radiotherapy. A report from the Intergroup Rhabdomyosarcoma Study (IRS) Committee.
abstract: OBJECTIVE
  Children with bladder rhabdomyosarcoma (RMS) are currently treated with primary chemotherapy and usually with local irradiation. More than 30% of this group ultimately require total cystectomy. The purpose of this study was to review the results of the use of partial as opposed to total cystectomy in 40 patients with bladder RMS during Intergroup Rhabdomyosarcoma Study (IRS)-I, -II, and -III (1972-1989).
  
  
  METHODS
  A total of 171 children with primary RMS of the bladder were enrolled in the IRS during this interval. Partial cystectomy was performed in 40, in 33 before any other therapy and in seven after 10-57 weeks of chemotherapy (primarily vincristine, actinomycin-D, and cyclophosphamide, i.e., VAC) +/- radiotherapy. Surviving patients have been observed for 4-22 years.
  
  
  RESULTS
  Thirty-one of 40 patients (78.5%) have been disease free for 2-16 years. Survival among all other IRS cases with bladder RMS during the same interval was 79.5%. Of the 31 surviving patients, one required secondary total cystectomy and two required bladder augmentation procedures for benign bladder contracture. Three quarters of the total group of living patients who have undergone partial cystectomy are without bladder-related symptoms or demonstrable lower urinary tract disease. The remaining patients have a history of functional bladder problems related to contracture or incontinence.
  
  
  CONCLUSIONS
  Partial cystectomy is an alternative to total cystectomy for bladder RMS when the tumor site makes it anatomically feasible. In such patients, it should be considered before total cystectomy and in patients with persistent areas of questionable residual tumor after otherwise apparently successful regimens of primary chemotherapy +/- radiotherapy.
authors.affiliation: Childrens Hospital Los Angeles, CA 90027, USA.
authors.forename: D M
authors.initials: DM
authors.lastname: Hays
authors.forename: R B
authors.initials: RB
authors.lastname: Raney
authors.forename: M D
authors.initials: MD
authors.lastname: Wharam
authors.forename: E
authors.initials: E
authors.lastname: Wiener
authors.forename: T E
authors.initials: TE
authors.lastname: Lobe
authors.forename: R J
authors.initials: RJ
authors.lastname: Andrassy
authors.forename: W
authors.initials: W
authors.lastname: Lawrence
authors.forename: J
authors.initials: J
authors.lastname: Johnston
authors.forename: B
authors.initials: B
authors.lastname: Webber
authors.forename: H M
authors.initials: HM
authors.lastname: Maurer
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D003609:Dactinomycin / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D001743:Urinary Bladder / Q000502:physiology; D001749:Urinary Bladder Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D003520:Cyclophosphamide