Beyhan BÜLBÜL, Murat ELLİ, Ayhan DAĞDEMİR, Nevzat DABAK, Sabri ACAR, Sancar BARIŞ, M.bekir SELÇUK, Gönül OĞUR

Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi

Bu çalışmada Çocuk Hematoloji-Onkoloji Bilim Dalı’nda takip ve tedavi edilen 27 Ewing Sarkomlu olgunun epidemiyolojik ve klinik özellikleri, tedavi sonuçları, uzun dönem komplikasyonları retrospektif olarak incelenmiştir. Olguların (n=27) ortalama tanı yaşı 11.36 ±3.98 yıl ( 8 ay- 17.9) olup, erkek/kız oranı 1.7 olarak saptandı. En sık başvuru yakınmalarının lokalize ağrı (%96.3) ve şişlik (%100) olduğu görüldü. Primer tümör bölgesi 16 olguda (%59.3) uzun kemiklerde, 11 olguda (%40.7) aksiyel kemiklerde saptandı. Primer lezyonun yerleştiği kemikler sırasıyla femur (%26), tibia (%14.8), pelvis (%14.8) ve fibula (%11.1) idi. Başvuru anında 3 olguda ( %11.1 ) kemik iliği tutulumu tespit edildi. Toplam 9 olguda (%33.3) tanı sırasında uzak yayılım saptandı. 23 olguya (%92) neoadjuvan kemoterapi, 17 olguya (%77.2) adjuvan kemoterapi uygulandığı tespit edildi. Olguların 13’üne ekstremite koruyucu cerrahi, 2’sine amputasyon yapıldığı saptandı. Radyoterapi olguların %65.3’ünde uygulandı (n=17) ve radyoterapi yeri 11 olguda primer tümör bölgesine, 5 olguda metastaz bölgesine, 1 olguda hem primer tümör hem metastaz bölgesine yönelik idi. Olgularının ortalama ve ortanca izlem süreleri sırasıyla 35.5 ve 19 ay (en fazla 355 ay) idi. Sağ kalım analizleri sonucunda toplam sağ kalım oranı %22.4±10.9 olarak saptandı. Retrospective assessment of patients with pediatric Ewing’s sarcoma In this study, 27 patients with Ewing’s sarcoma admitted to Pediatric Hematology and Oncology Department were evaluated retrospectively. The epidemiologic and clinical features, treatment results, long term side effects of therapy of 27 patients with Ewing’s sarcoma were analyzed. The mean age of patients with Ewing’s sarcoma (n=27) was 11.36 ±3.98 years (range,8 months-17.9 years) and male/female ratio was 1.7. The most common complaints were pain (96.3%) and swelling (100%). A total of 16 (59.3%) primary tumors were located in an extremity, whereas 11 (40.7%) occured in the axial skeleton. The most common primary lesion sites were femur (26%), tibia (14.8%), pelvis(14.8%) and fibula (11.1%) respectively. At diagnosis 3 patients (11.1%) had bone marrow metastases. A total of 9 patients (33.3%) had metastases at diagnosis. 23 patients (92%) received neoadjuvant chemotherapy and 17 patients received (77.2%) adjuvant chemotherapy. Limb salvage surgery was applied in 13 patients and amputation was made in 2 patients. Seventeen patients (65.3%) received radiotherapy; to the primary lesion site in 11 patients, the metastatic site in 5 patients, and both sites in one patient. The mean and median follow-up of patients with Ewing’s sarcoma was 35.5 and 19 months respectively (maximum 355 months). The overall survival rate was 22.4±10.9 % .

Keywords:

: Ewing sarkom, çocukluk çağı, retrospektif, analiz Key Words:Ewing’s sarcoma, childhood, retrospective, analyze,

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