European Journal of Pediatrics

, Volume 163, Issue 8, pp 452–456 | Cite as

Management of stage I cervical sarcoma botryoides in childhood and adolescence

  • Susanne E. M. Gruessner
  • Charles O. A. Omwandho
  • Thomas Dreyer
  • Renate Blütters-Sawatzki
  • Alfred Reiter
  • Hans R. Tinneberg
  • Rainer M. Bohle
Original Paper

Abstract

Rhabdomyosarcomas are the most common soft tissue sarcomas in childhood. The botryoid variant arises in infancy from the vagina or urinary bladder and extremely rarely from the uterine cervix. Treatment regimes range from local excision of the tumour to radical hysterectomy with adjuvant multidrug therapy and/or radiotherapy. In cases of minimal cervical invasion, the less invasive local excision in combination with adjuvant chemotherapy has resulted in excellent survival rates with complete functional preservation of the bladder, rectum, vagina, and ovaries. We present here a 30-year literature review and a case report of a cervical sarcoma botryoides in a 5-year-old girl. Conclusion:based on the literature review and our own observation, we recommend minor surgical approaches in combination with chemotherapy as the treatment of choice for early stage I cervical rhabdomyosarcoma.

Keywords

Cervix Chemotherapy Conservative surgery Embryonal rhabdomyosarcoma Sarcoma botryoides 

Abbreviations

IRS

Intergroup Rhabdomyosarcoma Study Group

RMS

rhabdomyosarcoma

VAC

vincristine, adriamycin, cyclophosphamide

VAI

vincristine, adriamycin, ifosfamide

References

  1. 1.
    Andrassy RJ (2002) Advances in the surgical management of sarcomas in children. Am J Surg 184: 484–491CrossRefPubMedGoogle Scholar
  2. 2.
    Andrassy RJ, Wiener ES, Raney RB, Hays DM, Arndt CAS, Lobe TE, Lawrence W, Anderson JR, Qualman SJ, Christ WM (1999) Progress in the surgical management of vaginal rhabdomyosarcoma: a 25-year review from the Intergroup Rhabdomyosarcoma Study Group. J Pediatr Surg 34: 731–735PubMedGoogle Scholar
  3. 3.
    Ashlock R, Johnstone PA (2003) Treatment modalities of bladder/prostate rhabdomyosarcoma review. Prostate Cancer Prostatic Dis 6: 112–120CrossRefPubMedGoogle Scholar
  4. 4.
    Baker KS, Anderson JR, Link MP et al (2000) Benefit of intensified therapy for patients with local or regional embryonal rhabdomyosarcoma: results from the Intergroup Rhabdomyosarcoma Study IV. J. Clin Oncol 18: 2427–2434Google Scholar
  5. 5.
    Balat O, Balat A, Verschraegen C, Tornos C, Edwards CL (1999) Sarcoma botryoides of the uterine endocervix: long-term results of conservative surgery. Eur J Gynaecol Oncol 17: 335–337Google Scholar
  6. 6.
    Brand E, Berek JS, Nieberg RK, Hacker NF (1987) Rhabdomyosarcoma of the uterine cervix: sarcoma botryoides. Cancer 60: 1552–1556PubMedGoogle Scholar
  7. 7.
    Copeland LJ, Gershenson DM, Saul PB, Sneige N, Stringer CA, Edwards CL (1985) Sarcoma botryoides of the female genital tract. Obstet Gynecol 66: 262–266PubMedGoogle Scholar
  8. 8.
    Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Flyer C, Hammond D, Hays DM, Hermann J, Heyn R et al (1995) The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 13: 610–630PubMedGoogle Scholar
  9. 9.
    Christ WM, Anderson JR, Meza JL et al (2001) Intergroup Rhabdomyosarcoma Study—IV: results for patients with nonmetastatic disease. J Clin Oncol 19: 3091–3102PubMedGoogle Scholar
  10. 10.
    Ferrari A, Casanova M, Bisogno G, Zanetti I, Cecchetto G, De Berna B, Riccardi R, Tamaro P, Meazza C, Alaggio R, Ninfo V, Carli M (2003) Rhabdomyosarcoma in infants younger than one year old: a report from the Italian Cooperative Group. Cancer 97: 2597–2604CrossRefPubMedGoogle Scholar
  11. 11.
    Flamant F, Rodary C, Rey A, Praquin MT, Sommelet D, Quintana E, Theobald S, Brunat-Mentigny M et al (1998) Treatment of non-metastatic rhabdomyosarcomas in childhood and adolescence. Results of the second study of the International Society of Paediatric Oncology: MMT84. Eur J Cancer 34: 1050–1062CrossRefPubMedGoogle Scholar
  12. 12.
    Ghavimi F, Exelby PR, D’Angio GJ (1975) Multidisciplinary treatment of embryonal rhabdomyosarcoma in children. Cancer 35: 677–686PubMedGoogle Scholar
  13. 13.
    Golbang P, Khan A, Scurry J, MacIsaac I, Planner R (1997) Cervical sarcoma botryoides and ovarian Sertoli-Leydig cell tumor. Gynecol Oncol 67: 102–106CrossRefPubMedGoogle Scholar
  14. 14.
    Gordon AN, Montag TW (1990) Sarcoma botryoides of the cervix: excision followed by adjuvant chemotherapy for preservation of reproductive function. Gynecol Oncol 36: 119–124CrossRefPubMedGoogle Scholar
  15. 15.
    Groff DB (2001) Pelvic neoplasms in children. J Surg Oncol 77: 65–71CrossRefPubMedGoogle Scholar
  16. 16.
    Hays DM, Shimada H, Raney RB, Teffet M, Newton W, Christ WM, Lawrence W, Ragab A, Maurer HM (1985) Sarcoma of the vagina and uterus: The Intergroup Rhabdomyosarcoma Study. J Pediatr Surg 20: 718–724PubMedGoogle Scholar
  17. 17.
    Herrera JM, Krebs A, Harris P, Barriga F (2000) Childhood tumours. Surg Clin North Am 80: 747–760PubMedGoogle Scholar
  18. 18.
    Hilgers RD (1975) Pelvic exenteration for vaginal embryonal rhabdomyosarcoma. A review. Obstet Gynecol 45: 175–180PubMedGoogle Scholar
  19. 19.
    Holton CP, Chapman KE, Lackey RW (1973) Extended combination therapy of childhood rhabdomyosarcoma. Cancer 32: 1310–1316PubMedGoogle Scholar
  20. 20.
    Ind T, Shepherd J (2003) Pelvic tumours in adolescence. Best Pract Res Clin Obstet Gynaecol 17: 149–168CrossRefPubMedGoogle Scholar
  21. 21.
    Martelli H, Oberlin O, ReyA, Godzinski J Spicer RD, Bouvet N, Haie-Meder C, Terrier-Lacombe MJ, Sanchez de Toledo J, Spooner D, Flammant F, Stevens MC (2000) Conservative treatment for girls with non-metastatic rhabdomyosarcoma of the genital tract: a report from the Study Committee of the International Society of Pediatric Oncology. J Clin Oncol 17: 2117–2122Google Scholar
  22. 22.
    Maurer HM (1988) The Intergroup Rhabdomyosarcoma Study-I. Cancer 61: 209–220PubMedGoogle Scholar
  23. 23.
    McCarville BM, Spunt SL, Pappo AS (2001) Rhabdomyosarcoma in pediatric patients: the good, the bad, and the unusual. AJR 176: 1563–1569Google Scholar
  24. 24.
    McDowell HP (2003) Update on childhood rhabdomyosarcoma. Arch Dis Child 88: 354–357CrossRefPubMedGoogle Scholar
  25. 25.
    Nag S, Tippin D, Ruymann FB (2003) Long-term morbidity in children treated with fractionated high-dose-rate brachytherapy for soft tissue sarcomas. J Pediatr Hematol Oncol 25: 448–452CrossRefPubMedGoogle Scholar
  26. 26.
    Ortega JA (1979) A therapeutic approach to childhood pelvic rhabdomyosarcoma of the uterine corpus and cervix. Gynecol Oncol 25: 171–194Google Scholar
  27. 27.
    Parham DL (1996) Rhabdomyosarcoma and related tumors. In: Parham DL (ed) Pediatric neoplasia: morphology and biology. Lippincott-Raven, Philadelphia, pp 87–104Google Scholar
  28. 28.
    Pastore G, Mosso ML, Carnevale F, di Montezemolo LC, Forni M, Madon E, Ricardi U, Terracini B, Magnani C (2001) Survival trends of childhood cancer diagnosed during 1970–1994 in Piedmont, Italy: a report from the Childhood Cancer Registry. Med Pediatr Oncol 35: 481–488CrossRefGoogle Scholar
  29. 29.
    Porzio G, Toro G, Paris I, Ricuveto E, Ficorella C, Marchetti P (2000) Cervical sarcoma botryoides treated with conservative surgery and adjuvant chemotherapy: a case report. Eur J Gynaecol Oncol 21: 499–500PubMedGoogle Scholar
  30. 30.
    Rutledge F, Sullivan M (1967) Sarcoma botryoides. Ann N Y Acad Sci 142: 694–708PubMedGoogle Scholar
  31. 31.
    Schalow EL, Broecker BH (2003) Role of surgery in children with rhabdomyosarcoma. Med Pediatr Oncol 41: 1-6CrossRefPubMedGoogle Scholar
  32. 32.
    Stiller CA, Stevens MCG, Magnani C, Corazziari I (2001) Survival of children with soft-tissue sarcoma in Europe since 1978: results from the EUROCARE study. Eur J Cancer 37: 767–774CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • Susanne E. M. Gruessner
    • 1
  • Charles O. A. Omwandho
    • 2
  • Thomas Dreyer
    • 3
  • Renate Blütters-Sawatzki
    • 4
  • Alfred Reiter
    • 4
  • Hans R. Tinneberg
    • 1
  • Rainer M. Bohle
    • 3
  1. 1.Department of Obstetrics and GynaecologyJustus-Liebig-University GiessenGiessen Germany
  2. 2.Department of BiochemistryUniversity of NairobiNairobi Kenya
  3. 3.Institute of PathologyJustus-Liebig-UniversityGiessen Germany
  4. 4.Department of Paediatric OncologyJustus-Liebig-UniversityGiessen Germany

Personalised recommendations