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Pediatric Surgery International

, Volume 29, Issue 10, pp 1077–1082 | Cite as

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

  • Tomoro HishikiEmail author
  • Takeshi Saito
  • Tetsuya Mitsunaga
  • Mitsuyuki Nakata
  • Elena Terui
  • Shugo Komatsu
  • Naoko Mise
  • Kazuaki Harada
  • Jun Iwai
  • Yasuyuki Higashimoto
  • Yuri Okimoto
  • Harumi Kakuda
  • Hidemasa Ochiai
  • Moeko Hino
  • Sumie Homma
  • Yuichi Osa
  • Hideo Yoshida
Original Article

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.

Keywords

Soft tissue sarcoma Rhabdomyosarcoma Children Surgery Pelvis 

References

  1. 1.
    Stevens MC (2005) Treatment for childhood rhabdomyosarcoma: the cost of cure. Lancet Oncol 6:77–84PubMedCrossRefGoogle Scholar
  2. 2.
    Raney RB, Maurer HM, Anderson JR, Andrassy RJ, Donaldson SS, Qualman SJ, Wharam MD, Wiener ES, Crist WM, The Intergroup Rhabdomyosarcoma Study Group (IRSG) (2001) Major lessons from the IRS-I through IRS-IV studies as background for the current IRS-V treatment protocols. Sarcoma 5:9–15. doi: 10.1080/13577140120048890 PubMedCrossRefGoogle Scholar
  3. 3.
    Malempati S, Hawkins DS (2012) Rhabdomyosarcoma: review of the Children’s Oncology Group (COG) Soft-Tissue Sarcoma Committee experience and the rationale fro current COG studies. Pediatr Blood Cancer 59:5–10PubMedCrossRefGoogle Scholar
  4. 4.
    Oberlin O, Rey A, Sanchez de Toledo J, Martelli H, Jenney ME, Scopinaro M, Bergeron C, Merks JH, Bouvet N, Ellershaw C, Kelsey A, Spooner D, Stevens MC (2012) Randomized comparison of intensified six-drug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapy-sensitive childhood soft tissue sarcomas: long-term results from the International Society of Pediatric Oncology MMT95 study. J Clin Oncol 30:2457–2465. doi: 10.1200/JCO.2011.40.3287 PubMedCrossRefGoogle Scholar
  5. 5.
    Meza JL, Anderson J, Pappo AS, Meyer WH, Children’s Oncology Group (2006) Analysis of prognostic factors in patients with nonmetastatic rhabdomyosarcoma treated on intergroup rhabdomyosarcoma studies III and IV: the Children’s Oncology Group. J Clin Oncol 24:3844–3851PubMedCrossRefGoogle Scholar
  6. 6.
    Stevens MC, Rey A, Bouvet N, Ellershaw C, Flamant F, Habrand JL, Marsden HB, Martelli H, Sanchez de Toledo J, Spicer RD, Spooner D, Terrier-Lacombe MJ, van Unnik A, Oberlin O (2005) Treatment of nonmetastatic rhabdomyosarcoma in childhood and adolescence: third study of the International Society of Paediatric Oncology—SIOP Malignant Mesenchymal Tumor 89. J Clin Oncol 23:2618–2628PubMedCrossRefGoogle Scholar
  7. 7.
    Filipas D, Fisch M, Stein R, Gutjahr P, Hohenfellner R, Thüroff JW (2004) Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery. BJU Int 93:125–129PubMedCrossRefGoogle Scholar
  8. 8.
    Raney RB, Stoner JA, Waltherhouse DO, Andrassy RJ, Donaldson SS, Laurie F, Meyer WH, Qualman SJ, Crist WM (2004) Results of Treatment of fifty-six patients with localized retroperitoneal and pelvic rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study-IV, 1991–1997. Pediatr Blood Cancer 42:618–625PubMedCrossRefGoogle Scholar
  9. 9.
    Rodeberg DA, Stoner JA, Hayes-Jordan A, Kao SC, Wolden SL, Qualman SJ, Meyer WH, Hawkins DS (2009) Prognostic significance of tumor response at the end of therapy in group III rhabdomyosarcoma: a report from the children’s oncology group. J Clin Oncol 27:3705–3711. doi: 10.1200/JCO.2008.19.5933 PubMedCrossRefGoogle Scholar
  10. 10.
    Seitz G, Dantonello TM, Int-Veen C, Blumenstock G, Godzinski J, Klingebiel T, Schuck A, Leuschner I, Koscielniak E, Fuchs J, CWS-96 Study Group (2011) Treatment efficiency, outcome and surgical treatment problems in patients suffering from localized embryonal bladder/prostate rhabdomyosarcoma: a report from the Cooperative Soft Tissue Sarcoma trial CWS-96. Pediatr Blood Cancer 56:718–724. doi: 10.1002/pbc.22950 PubMedCrossRefGoogle Scholar
  11. 11.
    Arndt C, Rodeberg D, Breitfeld PP, Raney RB, Ullrich F, Donaldson S (2004) Does bladder preservation (as a surgical principle) lead to retaining bladder function in bladder/prostate rhabdomyosarcoma? Results from intergroup rhabdomyosarcoma study IV. J Urol 171:2396–2403PubMedCrossRefGoogle Scholar
  12. 12.
    Alexander N, Lane S, Hitchcock R (2012) What is the evidence for radical surgery in the management of localized embryonal bladder/prostate rhabdomyosarcoma? Pediatr Blood Cancer 58:833–835PubMedCrossRefGoogle Scholar
  13. 13.
    Raney B, Stoner J, Anderson J, Andrassy R, Arndt C, Brown K, Crist W, Maurer H, Qualman S, Wharam M, Wiener E, Meyer W, Hayes-Jordan A, Soft-Tissue Sarcoma Committee of the Children’s Oncology Group (2010) Impact of tumor viability at second-look procedures performed before completing treatment on the Intergroup Rhabdomyosarcoma Study Group protocol IRS-IV, 1991–1997: a report from the children’s oncology group. J Pediatr Surg 45:2160–2168. doi: 10.1016/j.jpedsurg.2010.07.021 PubMedCrossRefGoogle Scholar
  14. 14.
    Merguerian PA, Agarwal S, Greenberg M, Bägli DJ, Khoury AE, McLorie GA (1998) Outcome analysis of rhabdomyosarcoma of the lower urinary tract. J Urol 160:1191–1194PubMedCrossRefGoogle Scholar
  15. 15.
    Yeung CK, Ward HC, Ransley PG, Duffy PG, Pritchard J (1994) Bladder and kidney function after cure of pelvic rhabdomyosarcoma in childhood. Br J Cancer 70:1000–1003PubMedCrossRefGoogle Scholar
  16. 16.
    Puri DR, Wexler LH, Meyers PA, La Quaglia MP, Healey JH, Wolden SL (2006) The challenging role of radiation therapy for very young children with rhabdomyosarcoma. Int J Radiat Oncol Biol Phys 65:1177–1184PubMedCrossRefGoogle Scholar
  17. 17.
    Spunt SL, Sweeney TA, Hudson MM, Billups CA, Krasin MJ, Hester AL (2005) Late effects of pelvic rhabdomyosarcoma and its treatment in female survivors. J Clin Oncol 23:7143–7151PubMedCrossRefGoogle Scholar
  18. 18.
    Breneman J, Meza J, Donaldson SS, Raney RB, Wolden S, Michalski J, Laurie F, Rodeberg DA, Meyer W, Walterhouse D, Hawkins DS (2011) Local control with reduced-dose radiotherapy for low-risk rhabdomyosarcoma: a report from the Children’s Oncology Group D9602 study. Int J Radiat Oncol Biol Phys 83:720–726. doi: 10.1016/j.ijrobp.2011.06.2011 PubMedGoogle Scholar
  19. 19.
    Rodeberg DA, Anderson JR, Arndt CA, Ferrer FA, Raney RB, Jenney ME, Brecht IB, Koscielniak E, Carli M, Bisogno G, Oberlin O, Rey A, Ullrich F, Stevens MC, Meyer WH (2011) Comparison of outcomes based on treatment algorithms for rhabdomyosarcoma of the bladder/prostate: combined results from the Children’s Oncology Group, German Cooperative Soft Tissue Sarcoma Study, Italian Cooperative Group, and International Society of Pediatric Oncology Malignant Mesenchymal Tumors Committee. Int J Cancer 128:1232–1239. doi: 10.1002/ijc.25444 PubMedCrossRefGoogle Scholar
  20. 20.
    Ferrer FA, Isakoff M, Koyle MA (2006) Bladder/prostate rhabdomyosarcoma: past, present and future. J Urol 176:1283–1291PubMedCrossRefGoogle Scholar
  21. 21.
    Macedo A Jr, Ferreira PV, Barroso U Jr, Demarchi GT, Garrone G, Liguori R, Caran E, Ortiz V (2010) Sexual function in teenagers after multimodal treatment of pelvic rhabdomyosarcoma: a preliminary report. J Pediatr Urol 6:605–608. doi: 10.1016/j.jpurol.2010.06.001 PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Tomoro Hishiki
    • 1
    • 2
    Email author
  • 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. 1.Department of Pediatric SurgeryChiba University Graduate School of MedicineChuo-kuJapan
  2. 2.Department of Pediatric SurgeryChiba Children’s HospitalMidori-kuJapan
  3. 3.Department of Hematology and OncologyChiba Children’s HospitalMidori-kuJapan
  4. 4.Department of PediatricsChiba University Graduate School of MedicineChuo-kuJapan
  5. 5.Department of UrologyChiba Children’s HospitalMidori-kuJapan

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