Öz
Thirty-one patients with non-metastatic rhabdomyosarcoma treated at Pediatric Oncology Department between 1994-2007 were reviewed retrospectively. Median age of the patients was 7. Male/female ratio was 4.1. Extremities were the most common location sites. Embryonal type was the most common histology. According to Intergroup Rhabdomyosarcoma Study Clinical Grouping system, 29% of the patients were group 1,29% of the patients were Group 2 and 42% of the patients were Group 3. Complete resection was performed at 48.4% of the patients. Seventeen patients were treated with EVAIA chemotherapy regimen, eight of them with VACA and the rest were treated with other regimens. Five-year event free survival and overall survival rates were 50% and 67%. Although most of our patients had poor prognostic location sites, the survival rates are comparable with developed countries.
Anahtar Kelimeler
Kaynakça
- 1. Lanzkowsky P, Rhabdomyosarcoma and Other Soft Tissue Sarcomas. In: Pediatric Hematology and Oncology; Third edition. San Diego: Academic Press; 2000:527-553.
- 2. Wexler LH, Crist WM. Rhabdomyosarcoma and the undifferantiated sarcomas. In Pizzo PA, Poplack DG(ed). Fourth edition. Principles and Practise of Pediatric Oncology Philadelphia: Lippincott Williams & Wilkins; 2002:939-971.
- 3. Crist WM, Anderson JR, Meza JL et al. Intergroup rhabdomyosarcoma study IV:Results for patients with non-metastatic disease. J Clin Oncology 2001;19:3091-3102.
- 4. Meza JL, Anderson JR, Pappo AS et al. Analysis of prognostic factors in patients with nonmetastatic rhabdomyosarcoma treated on intergroup rhabdomyosarcoma studies III and IV: The Children’s Oncology Group. J Clin Oncology 2006;24:3844-3851.
- 5. Stevens MC, Rey A, Bouvet N et al. Treatment of nonmetastatic rhabdomyosarcoma in childhood and adolescence: Third study of the international society of paediatric oncology-SIOP Malignant Mesenchymal Tumor 89. J Clin Oncology 2005;23:2618-2628.
- 6. Akyüz C, Sancak R, Büyükpamukçu N et al. Turkish experience with rhabdomyosarcoma: an analysis of 255 patients for 20 years. Turk J Pediatr 1998;40:491-501.
- 7. Koscielniak E, Harms D, Henze G et al. Results of treatment for soft tissue sarcoma in childhood and adolescence: A final report of the German Cooperative Soft Tissue Sarcoma Study CWS-86. J Clin Oncology 1999;17:3706-3719.
Öz
Merkezimize 1994-2007 yılları arasında başvuran otuz bir non-metastatik rabdomiy-osarkomlu hasta retrospektif olarak değerlendirildi. Hastalar; genel özellikleri ve sağkalım sonuçları açısından incelenerek literatürdeki serilerle karşılaştırıldı. Hastaların median yaşı 7, erkek/kız oranı 4.1 bulundu. En sık ekstremite yerleşimli tümörler görülürken, en sık rastlanan tümör histopatolojisi %51.6 ile embriyonel tip idi. Tümör evresi olarak %29 hasta İRS Grup 1, %29 hasta Grup 2 ve %42 hasta Grup 3’te yer aldı. Tanı anında %48.4 hastada tam ve tama yakın rezeksiyon gerçekleştirildi. Onyedi hasta EVAIA protokolü, sekiz hasta VACA protokolü ve altı hasta diğer protokoller ile tedavi edildi. Hastaların beş yıllık olaysız (EFS) ve genel yaşam hızları (OS) %50 ve %67 bulundu. Hastalarımızda genellikle kötü yerleşimli tümörler mevcut olmasına rağmen, elde edilen yaşam hızları gelişmiş ülkeler ile karşılaştırılabilir düzeydedir.
Dr. Neriman SARP,
Dr. Tuba EREN,
Dr. Şule YEŞİL,
Dr. İnci Ergürhan İLHAN
Anahtar Kelimeler
Kaynakça
- 1. Lanzkowsky P, Rhabdomyosarcoma and Other Soft Tissue Sarcomas. In: Pediatric Hematology and Oncology; Third edition. San Diego: Academic Press; 2000:527-553.
- 2. Wexler LH, Crist WM. Rhabdomyosarcoma and the undifferantiated sarcomas. In Pizzo PA, Poplack DG(ed). Fourth edition. Principles and Practise of Pediatric Oncology Philadelphia: Lippincott Williams & Wilkins; 2002:939-971.
- 3. Crist WM, Anderson JR, Meza JL et al. Intergroup rhabdomyosarcoma study IV:Results for patients with non-metastatic disease. J Clin Oncology 2001;19:3091-3102.
- 4. Meza JL, Anderson JR, Pappo AS et al. Analysis of prognostic factors in patients with nonmetastatic rhabdomyosarcoma treated on intergroup rhabdomyosarcoma studies III and IV: The Children’s Oncology Group. J Clin Oncology 2006;24:3844-3851.
- 5. Stevens MC, Rey A, Bouvet N et al. Treatment of nonmetastatic rhabdomyosarcoma in childhood and adolescence: Third study of the international society of paediatric oncology-SIOP Malignant Mesenchymal Tumor 89. J Clin Oncology 2005;23:2618-2628.
- 6. Akyüz C, Sancak R, Büyükpamukçu N et al. Turkish experience with rhabdomyosarcoma: an analysis of 255 patients for 20 years. Turk J Pediatr 1998;40:491-501.
- 7. Koscielniak E, Harms D, Henze G et al. Results of treatment for soft tissue sarcoma in childhood and adolescence: A final report of the German Cooperative Soft Tissue Sarcoma Study CWS-86. J Clin Oncology 1999;17:3706-3719.
Ayrıntılar
| Birincil Dil | Türkçe |
|---|---|
| Konular | Bebek ve Çocuk Sağlığı |
| Bölüm | Araştırma Makalesi |
| Yazarlar | |
| Yayımlanma Tarihi | 20 Nisan 2011 |
| Yayımlandığı Sayı | Yıl 2011 Cilt: 5 Sayı: 1 |
