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

Abstract

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.

Keywords

• Orbita, rabdomyosarkom

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