title: Optimal surgical treatment and urological outcomes in boys with pelvic and urogenital rhabdomyosarcomas and soft tissue sarcomas.
abstract: BACKGROUND
  Soft tissue sarcomas (STS) of pelvic origin in boys often involve the urogenital organs. The optimal extensiveness of radical surgery has long been an issue of discussion, since exenterative surgeries result in severe urogenital adverse effects. We conducted a retrospective review of patients with pelvic STS treated in two regional center hospitals and assessed the radicality of surgery and the functional outcome of the bladder.
  
  
  METHODS
  Medical records and surgical reports of nine cases (embryonal rhabdomyosarcoma 6, malignant triton tumor 2, suspected rhabdomyosarcoma 1) treated within 1997-2012 were reviewed. Site of origin was prostate in seven, retroperitoneal in two. Average follow-up period was 96 months.
  
  
  RESULTS
  All cases were subjected to neoadjuvant chemotherapy. Response was PR in four, SD in two, and PD in two. Radical surgery resulted in gross total resection in eight, and partial resection in one. Three underwent cystoprostatectomy, two cases underwent prostatectomy, and bladder-preserving tumor resection was carried out in four cases. At the last follow-up, three retained a functional bladder. Two required augmentation cystoplasty with intestinal conduits.
  
  
  CONCLUSIONS
  The majority of the on-going clinical trials recommend conservative surgery for bladder/prostate rhabdomyosarcoma, and to preserve the bladder function particularly in chemosensitive tumors. Some other groups, however, advocate the importance of radical surgery to prevent local relapse. These reports include heterogenous group of patients in the cohort, and therefore it is difficult to draw a conclusion of which approach truly contributes to the survival of the patients better. Future studies should evaluate bladder and sexual function objectively to establish reliable evidence regarding the benefit and adverse effects of different surgical approaches. These data would be informative to optimize the treatment balance for children with pelvic rhabdomyosarcomas.
authors.affiliation: Department of Pediatric Surgery, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8677, Japan, hishiki-tmr@umin.ac.jp.
authors.forename: Tomoro
authors.initials: T
authors.lastname: Hishiki
authors.forename: Takeshi
authors.initials: T
authors.lastname: Saito
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Mitsunaga
authors.forename: Mitsuyuki
authors.initials: M
authors.lastname: Nakata
authors.forename: Elena
authors.initials: E
authors.lastname: Terui
authors.forename: Shugo
authors.initials: S
authors.lastname: Komatsu
authors.forename: Naoko
authors.initials: N
authors.lastname: Mise
authors.forename: Kazuaki
authors.initials: K
authors.lastname: Harada
authors.forename: Jun
authors.initials: J
authors.lastname: Iwai
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Higashimoto
authors.forename: Yuri
authors.initials: Y
authors.lastname: Okimoto
authors.forename: Harumi
authors.initials: H
authors.lastname: Kakuda
authors.forename: Hidemasa
authors.initials: H
authors.lastname: Ochiai
authors.forename: Moeko
authors.initials: M
authors.lastname: Hino
authors.forename: Sumie
authors.initials: S
authors.lastname: Homma
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Osa
authors.forename: Hideo
authors.initials: H
authors.lastname: Yoshida
mesh_terms: D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D010386:Pelvic Neoplasms / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D012509:Sarcoma / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D016896:Treatment Outcome; D014554:Urination / Q000502:physiology*; D014565:Urogenital Neoplasms / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D013519:Urogenital Surgical Procedures / Q000379:methods*