The results of treatment of urogenital system rhabdomyosarcoma in children. 15-years of experience

Background. Despite significount successes in treatment of rhabdomyosarcoma of urogenital system in children there are unresolved questions of choise of optimal chemotherapys combinations, intensity of chemotherapy, volumes and terms of radiotherapy, tactics of treatment residual tumors in last 3 decades.

The objective: show 15 years experience of treatment local and locally prevalent rhabdomyosarcoma urogenital system in children. The prognosis for children and adolescents with rhabdomyosarcoma has improved with refinements in multi-modal therapy.

Materials and methods. In reseach are included 86 patients with a median age of 8.4 (0.7–17) with a local genitourinary rhabdomyosarcoma, treated in N.N. Blokhin National Medical Research Centre of Oncology from 2000 to 2016. All patients were treated in different riskadopted clinical protocol included chemotherapy and radiotherapy (IRS, SIOP, CWS and local protocol DORMS-6).

Results. A 10-year overall survival and disease-free survival rates were 76 and 72 % in the entire group rhabdomyosarcoma patients, respectively.

Conclusion. The effectiveness of the risk-adopted strategy in the genitourinary rhabdomyosarcoma treatment as well as the need of new approaches and in the cases of residual viable tumor after induction chemotherapy was demonstrated.

1. Hawkins D.S., Spunt S.L., Skapek S.X. COG Soft Tissue Sarcoma Committee. Children’s Oncology Group’s 2013 blueprint for research: Soft tissue sarcomas. Pediatr Blood Cancer 2013;60(6):1001–8. DOI: 10.1002/pbc.24435.

2. Ritchey M.L., Shamberger R.C. Pediatric Urologic Oncology. In: Campbell-Walsh Urology. 10th edn. Ed.: A.J. Wein. Philadelphia: Elsevier Saunders, 2012. Pp. 3704–9.

3. Meza J.L., Anderson J., Pappo A.S. et al. Analysis of prognostic factors in patients with nonmetastatic rhabdomyosarcoma treated on Intergroup Rhabdomyosarcoma Studies III and IV. J Clin Oncol 2006;24(24):3844–51. DOI: 10.1200/JCO.2005.05.3801.

4. Maurer H.M., Beltangady M., Gehan E.A. et al. The Intergroup Rhabdomyosarcoma Study-I. A final report. Cancer 1988;61(2):209–20.

5. Crist W.M., Anderson J.R., Meza J.L. et al. Intergroup rhabdomyosarcoma study-iv: results for patients with nonmetastatic disease. J Clin Oncol 2001;19(12):3091–102. DOI: 10.1200/JCO.2001.19.12.3091.

6. Baker K.S., Anderson J.R., Link M.P. et al. Benefit of intensified therapy for patients with local or regional embryonal rhabdomyosarcoma: results from the Intergroup Rhabdomyosarcoma Study IV. J Clin Oncol 2000;18(12):2427–34. DOI: 10.1200/JCO.2000.18.12.2427.

7. Raney R.B. Jr, Gehan E.A., Hays D.M. et al. Primary chemotherapy with or without radiation therapy and/or surgery for children with localized sarcoma of the bladder, prostate, vagina, uterus, and cervix. A comparison of the results in Intergroup Rhabdomyosarcom Studies I and II. Cancer 1990;66(10):2072–81.

8. Raney B., Anderson J., Jenney M. et al. Late effects in 164 patients with rhabdomyosarcoma of the bladder/prostate region: a report from the International Workshop. J Urol 2006;176(5):2190–5. DOI: 10.1016/j.juro.2006.07.064.

9. Seitz G., Dantonello T.M., Int-Veen C. et al. 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 2011;56(5):718–24. DOI: 10.1002/pbc.22950.

10. Oberlin O., Rey A., de Toledo J.S. et al. Randomized comparison of intensified sixdrug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapysensitive childhood soft tissue sarcomas: long-term results from the International Society of Pediatric Oncology MMT95 study. J Clin Oncol 2012;30(20):2457–65. DOI: 10.1200/JCO.2011.40.3287.

11. Raney B., Stoner J., Anderson J. et al. Impact of tumor viability at second-look procedure before completing treatment on the Intergroup Rhabdomyosarcoma Study Group protocol IRS-IV 1991–97: a report from the Children’s Oncology Group. J Pediatr Surg 2010;45:2160–8.