The treatment results of orbital rhabdomyosarcoma: three case reports

Abstract

Rhabdomyosarcoma (RMS) is the most common malignant soft-tissue tumor in childhood. The orbit is the primary site in 9-10% of these tumors. In childhood orbital RMS, the 5-year overall survival rates have increased to the rates of 85- 90% with the use of multidisciplinary approach. In this study, the early results of two males and one female patients treated with ERT in our clinic were evaluated. While two patients received external radiotherapy after induction chemotherapy, the other one received concomittantly. ERT was given to all patients in a fraction size of 1.6-1.8 Gy per day to a total dose of 41.4-50.4 Gy. After ERT, in a short period of time, symptomatic and radiologic response were achieved in all patients. External radiotherapy plays a critical role in maintaining a sustainable long term local control and rapid symptomatic relief. To maintain the succesful results without degrading the quality of life in childhood orbital RMS; modern computerized planning systems, 3-D conformal radiotherapy, intensity modulated radiotherapy and proton therapy are being used.

Keywords

• Radiotherapy,Rhabdomyosarcoma,Orbital Neoplasms,Child,Child, Preschool,Male,Female,Chemotherapy,Treatment Outcome

Orbital rabdomyosarkomlarda tedavi sonuçları: Üç olgu sunumu

Öz

Rabdomyosarkomlar (RMS) çocukluk çağında en sık görülen yumuşak doku sarkomlarıdır. Rabdomyosarkomların %9- 10'u orbital yerleşimlidir. Çocukluk çağı orbital RMS'lerinde multidisipliner tedavi yaklaşımlarıyla sağkalım oranları %85- 90'lara yükselmiştir. Bu yazıda orbital rabdomyosarkom tanısıyla kliniğimizde eksternal radyoterapi (RT) uygulanan iki erkek, bir kız hastanın erken tedavi sonuçları değerlendirildi. İki hastaya indüksiyon kemoterapisi (KT) sonrası, bir hastaya ise biyopsi sonrası KT ile eşzamanlı olarak eksternal RT uygulandı. Hastalara 1.6-1.8 Gy/gün fraksiyon dozuyla toplam 41.4-50.4 Gy eksternal RT verildi. RT'den sonra kısa zamanda hastalarda semptomatik ve radyolojik yanıt elde edildi. Eksternal RT lokal hastalık kontrolünün kalıcı sağlanması ve hızlı semptomatik cevap açısından kritik öneme sahiptir. Çocukluk çağı orbital RMS'lerde yaşam kalitesini bozmadan tedavi başarısının sağlanmasında gelişmiş bilgisayarlı planlama sistemleri, üç boyutlu konformal RT, yoğunluk ayarlı radyoterapi (IMRT) ve proton tedavileri kullanılmaktadır.

Anahtar Kelimeler

• Orbita, rabdomyosarkom

Kaynakça

1. 1. Wexler LH, Crist WM, Hilman LJ. Rhabdomyosarcoma and the undifferentiated sarcomas. In: Pizzo PA, Poplack DG, editors. Principles and practices of pediatric oncology. Philadelphia: Lippincott Williams&Wilkins; 2001. p. 939-71.
2. 2. Pizzo P. Rhabdomyosarcoma and the soft tissue sarcomas. In: Levine A, editor. Cancer in the young. New York: Masson; 1982. p. 615-32.
3. 3. Caillaud JM, Gerard-Marchant R, Marsden HB, van Unnik AJ, Rodary C, Rey A, et al. Histopathological classification of childhood rhabdomyosarcoma: a report from the International Society of Pediatric Oncology pathology panel. Med Pediatr Oncol 1989;17(5):391-400.
4. 4. Maurer HM, Gehan EA, Beltangady M, Crist W, Dickman PS, Donaldson SS, et al. The Intergroup Rhabdomyosarcoma Study-II. Cancer 1993;71(5):1904-22.
5. 5. Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C, et al. The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 1995;13(3):610-30.
6. 6. Fiorillo A, Migliorati R, Grimaldi M, Vassallo P, Canale G, Tranfa F, et al. Multidisciplinary treatment of primary orbital rhabdomyosarcoma. A single-institution experience. Cancer 1991;67(3):560-3.
7. 7. Shields JA, Shields CL. Rhabdomyosarcoma: review for the ophthalmologist. Surv Ophthalmol 2003;48(1):39-57.
8. 8. Crist WM, Garnsey L, Beltangady MS, Gehan E, Ruymann F, Webber B, et al. Prognosis in children with rhabdomyosarcoma: a report of the intergroup rhabdomyosarcoma studies I and II. Interg r o u p Rhabdomyosarcoma Committee. J Clin Oncol 1990;8(3):443-52.

9. 9. Rousseau P, Flamant F, Quintana E, Voute PA, Gentet JC. Primary chemotherapy in rhabdomyosarcomas and other malignant mesenchymal tumors of the orbit: results of the International Society of Pediatric Oncology MMT 84 Study. J Clin Oncol 1994;12(3):516-21.
10. 10. Oberlin O, Rey A, Anderson J, Carli M, Raney RB, Treuner J, et al. Treatment of orbital rhabdomyosarcoma: survival and late effects of treatment--results of an international workshop. J Clin Oncol 2001;19(1):197- 204.
11. 11. Lawrence W Jr, Gehan EA, Hays DM, Beltangady M, Maurer HM. Prognostic significance of staging factors of the UICC staging system in childhood rhabdomyosarcoma: a report from the Interg r o u p Rhabdomyosarcoma Study (IRS-II). J Clin Oncol 1987;5(1):46-54.
12. 12.Rodary C, Gehan EA, Flamant F, Treuner J, Carli M, Auquier A, et al. Prognostic factors in 951 nonmetastatic rhabdomyosarcoma in children: a report from the International Rhabdomyosarcoma Workshop. Med Pediatr Oncol 1991;19(2):89-95.
13. 13.Raney RB, Anderson JR, Barr FG, Donaldson SS, Pappo AS, Qualman SJ, et al. Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V. J Pediatr Hematol Oncol 2001;23(4):215-20.
14. 14. Punyko JA, Mertens AC, Baker KS, Ness KK, Robison LL, Gurney JG. Long-term survival probabilities for childhood rhabdomyosarcoma. A population-based evaluation. Cancer 2005;103(7):1475-83.
15. 15. Maurer HM, Beltangady M, Gehan EA, Crist W, Hammond D, Hays DM, et al. The Interg r o u p Rhabdomyosarcoma Study-I. A final report. Cancer 1988;61(2):209-20.
16. 16.Baker KS, Anderson JR, Link MP, Grier HE, Qualman SJ, Maurer HM, et al. Benefit of intensified therapy for patients with local or regional embryonal rhabdomyosarcoma: results from the Interg r o u p Rhabdomyosarcoma Study IV. J Clin Oncol 2000;18(12):2427-34.
17. 17. Lawrence W Jr, Anderson JR, Gehan EA, Maurer H. Pretreatment TNM staging of childhood rhabdomyosarcoma: a report of the Interg r o u p Rhabdomyosarcoma Study Group. Children's Cancer Study Group. Pediatric Oncology Group. Cancer 1997;80(6):1165-70.
18. 18. Wharam M, Beltangady M, Hays D, Heyn R, Ragab A, Soule E, et al. Localized orbital rhabdomyosarcoma. An interim report of the Interg r o u p Rhabdomyosarcoma Study Committee. Ophthalmology 1987;94(3):251-4.
19. 19. Heyn R, Ragab A, Raney RB Jr, Ruymann F, Tefft M, Lawrence W Jr, et al. Late effects of therapy in orbital rhabdomyosarcoma in children. A report from the I n t e rgroup Rhabdomyosarcoma Study. Cancer 1986;57(9):1738-43.
20. 20.Raney RB, Anderson JR, Kollath J, VassilopoulouSellin R, Klein MJ, Heyn R, et al. Late effects of therapy in 94 patients with localized rhabdomyosarcoma of the orbit: Report from the Interg r o u p Rhabdomyosarcoma Study (IRS)-III, 1984-1991. Med Pediatr Oncol 2000;34(6):413-20.
21. 21. Miralbell R, Cella L, Weber D, Lomax A. Optimizing radiotherapy of orbital and paraorbital tumors: intensity-modulated X-ray beams vs. intensity-modulated proton beams. Int J Radiat Oncol Biol Phys 2000;47(4):1111-9.
22. 22. Hug EB, Sweeney RA, Nurre PM, Holloway KC, Slater JD, Munzenrider JE. Proton radiotherapy in management of pediatric base of skull tumors. Int J Radiat Oncol Biol Phys 2002;52(4):1017-24.
23. 23.Cassady JR, Sagerman RH, Tretter P, Ellsworth RM. Radiation therapy for rhabdomyosarcoma. Radiology 1968;91(1):116-20.
24. 24. Wharam MD, Hanfelt JJ, Te fft MC, Johnston J, Ensign LG, Breneman J, et al. Radiation therapy for rhabdomyosarcoma: local failure risk for Clinical Group III patients on Intergroup Rhabdomyosarcoma Study II. Int J Radiat Oncol Biol Phys 1997;38(4):797-804.
25. 25. Kodet R, Newton WA Jr, Hamoudi AB, Asmar L, Wharam MD, Maurer HM. Orbital rhabdomyosarcomas and related tumors in childhood: relationship of morphology to prognosis-an Interg r o u p Rhabdomyosarcoma study. Med Pediatr Oncol 1997;29(1):51-60.
26. 26. Notis CM, Abramson DH, Sagerman RH, Ellsworth RM. Orbital rhabdomyosarcoma: treatment or overtreatment. Ophthalmic Genet 1995;16(4):159-62.
27. 27. Donaldson SS, Asmar L, Breneman J, Fryer C, Glicksman AS, Laurie F, et al. Hyperfractionated radiation in children with rhabdomyosarcoma-results of an Intergroup Rhabdomyosarcoma Pilot Study. Int J Radiat Oncol Biol Phys 1995;32(4):903-11.
28. 28. Donaldson SS, Meza J, Breneman JC, Crist WM, Laurie F, Qualman SJ, et al. Results from the IRS-IV randomized trial of hyperfractionated radiotherapy in children with rhabdomyosarcoma-a report from the IRSG. Int J Radiat Oncol Biol Phys 2001;51(3):718- 28.
29. 29.Regine WF, Fontanesi J, Kumar P, Ayers D, Bowman LC, Pappo AS, et al. Local tumor control in rhabdomyosarcoma following low-dose irradiation: comparison of group II and select group III patients. Int J Radiat Oncol Biol Phys 1995;31(3):485-91.