Optimal surgical treatment and urological outcomes in boys with pelvic and urogenital rhabdomyosarcomas and soft tissue sarcomas

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• 作者：Tomoro Hishiki ; Takeshi Saito ; Tetsuya Mitsunaga…
• 关键词：Soft tissue sarcoma ; Rhabdomyosarcoma ; Children ; Surgery ; Pelvis
• 刊名：Pediatric Surgery International
• 出版年：2013
• 出版时间：October 2013
• 年：2013
• 卷：29
• 期：10
• 页码：1077-1082
• 全文大小：216KB
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• 作者单位：Tomoro Hishiki (1) (2)
  Takeshi Saito (1)
  Tetsuya Mitsunaga (1)
  Mitsuyuki Nakata (1)
  Elena Terui (1)
  Shugo Komatsu (1) (2)
  Naoko Mise (1)
  Kazuaki Harada (1)
  Jun Iwai (2)
  Yasuyuki Higashimoto (2)
  Yuri Okimoto (3)
  Harumi Kakuda (3)
  Hidemasa Ochiai (4)
  Moeko Hino (4)
  Sumie Homma (5)
  Yuichi Osa (5)
  Hideo Yoshida (1)
  
  1. Department of Pediatric Surgery, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8677, Japan
  2. Department of Pediatric Surgery, Chiba Children’s Hospital, 579-1 Heta-cho, Midori-ku, Chiba, 266-0077, Japan
  3. Department of Hematology and Oncology, Chiba Children’s Hospital, 579-1 Heta-cho, Midori-ku, Chiba, 266-0077, Japan
  4. Department of Pediatrics, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8677, Japan
  5. Department of Urology, Chiba Children’s Hospital, 579-1 Heta-cho, Midori-ku, Chiba, 266-0077, Japan
  
• ISSN：1437-9813

文摘

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. Patients Medical records and surgical reports of nine cases (embryonal rhabdomyosarcoma 6, malignant triton tumor 2, suspected rhabdomyosarcoma 1) treated within 1997-012 were reviewed. Site of origin was prostate in seven, retroperitoneal in two. Average follow-up period was 96?months. Treatment and outcome 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.