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

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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.

Patients

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.

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.

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Authors and Affiliations

  1. Department of Pediatric Surgery, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8677, Japan

    Tomoro Hishiki, Takeshi Saito, Tetsuya Mitsunaga, Mitsuyuki Nakata, Elena Terui, Shugo Komatsu, Naoko Mise, Kazuaki Harada & Hideo Yoshida

  2. Department of Pediatric Surgery, Chiba Children’s Hospital, 579-1 Heta-cho, Midori-ku, Chiba, 266-0077, Japan

    Tomoro Hishiki, Shugo Komatsu, Jun Iwai & Yasuyuki Higashimoto

  3. Department of Hematology and Oncology, Chiba Children’s Hospital, 579-1 Heta-cho, Midori-ku, Chiba, 266-0077, Japan

    Yuri Okimoto & Harumi Kakuda

  4. Department of Pediatrics, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8677, Japan

    Hidemasa Ochiai & Moeko Hino

  5. Department of Urology, Chiba Children’s Hospital, 579-1 Heta-cho, Midori-ku, Chiba, 266-0077, Japan

    Sumie Homma & Yuichi Osa

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  1. Tomoro Hishiki
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  2. Takeshi Saito
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  3. Tetsuya Mitsunaga
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  4. Mitsuyuki Nakata
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  5. Elena Terui
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  6. Shugo Komatsu
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  7. Naoko Mise
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  11. Yuri Okimoto
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  15. Sumie Homma
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  16. Yuichi Osa
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  17. Hideo Yoshida
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Correspondence to Tomoro Hishiki.

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Hishiki, T., Saito, T., Mitsunaga, T. et al. Optimal surgical treatment and urological outcomes in boys with pelvic and urogenital rhabdomyosarcomas and soft tissue sarcomas. Pediatr Surg Int 29, 1077–1082 (2013). https://doi.org/10.1007/s00383-013-3400-y

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