Publication: Ewing Sarkom Tanısı İle İzlenen Çocuk Hastaların Retrospektif Değerlendirilmesi
Abstract
Amaç: Ewing sarkomu (ES) kemiğin primer malign tümörlerindendir. Çoğu çocukluk çağı ve adolesan yaş grubunda görülür ve prognozu genellikle kötüdür. Tedavisinde cerrahi, kemoterapi/kök hücre nakli ve radyoterapi kullanılmaktadır. Çalışmamızda takip ve tedavisi kliniğimizde yapılan hastaların demografik ve hastalık verilerini, tedavi sonuçlarını ve prognostik faktörlerin sağ kalım ile ilişkisini incelemeyi amaçladık. Hastalar ve Yöntem: Retrospektif nitelikteki çalışmamıza Ondokuz Mayıs Üniversitesi Tıp Fakültesi Hastanesi Çocuk Onkoloji Kliniğine 1 Ocak 2000-1 Aralık 2021 tarihleri arasında başvuran ve ES tanısı alan 80 hasta dahil edildi; tedavi sonuçları ve prognostik faktörlerin değerlendirilmesinde ise tanı, takip ve tedavisini sadece kliniğimizde devam ettiren 71 hasta dahil edildi. Hastaların yaş, cinsiyet, başvuru şikâyeti, laboratuvar değerleri, tümör yerleşim ve hacmi, tanı anındaki metastaz durumları, neoadjuvan tedavi protokolleri, uygulanan cerrahi, tümör dokusunun neoadjuvan tedaviye verdiği yanıt, adjuvan tedavi protokolleri, takipte gelişen nüks ve metastazlar, uygulanan tedavilerin akut ve geç yan etkileri ve bu verilerin sağ kalım ile ilişkisi incelendi. p <0,05 değeri istatistiki olarak anlamlı kabul edildi. Bulgular: Vakalarımızın ortalama tanı yaşının 14,1 ± 7,6, erkek/kadın oranı 1,2 en sık başvuru şikâyetinin şişlik (%74,6) ve ağrı (%69), ekstremite yerleşiminin daha sık (%52) olduğu, tüm vakalar arasında kemikte en sık tümör yerleşiminin femur (%18,3) olduğu saptandı. Vakalarımızn %29,5'inde tanı anında metastatik tutulum mevcuttu. Vakalarımızın %27,5'nde başvuru LDH düzeyinin 500 IU/L üzeri ve %30'unda tümör hacminin 100 ml'den fazla olduğu görüldü. Hastaların %64,7'sine neoadjuvan kemoterapi protokolü, %84,5'ine cerrahi uygulandı. Cerrahi tedavilerin ağırlıklı kısmını tümör rezeksiyonu oluşturmaktaydı. Vakaların %26,7'sine otolog kök hücre tedavisi uygulandı. Hastaların tamamında uygulanan tedavilere bağlı değişen dercelerde akut yan etki, %45'inde ise geç yan etki görülmüştür. Çalışma yapıldığı sırada hastaların %50'si kaybedilmişken %40'ı hayatına hastalıksız olarak devam etmekte idi; %7,5'u tedavisine devam etmekteyken % 2,5 olgunun son verilerine ulaşılamamıştır. Sonuçlar ve Öneriler: Çalışmamızda tanı anında başvuru yaşının 10 yaş ve üzeri olması, LDH değerinin 500 IU/L üzerinde olması, neoadjuvan KT'ye tümör dokusunun verdiği histopatolojik yanıt, otolog kök hücre nakli yapılmaması, tümör volümünün 100 ml ve üzerinde olması ve tanıda insizyonel biyopsinin kullanılmasının sağkalımı istatistiksel olarak anlamlı derecede düşürdüğü gösterilmiştir. Çoklu değişken analizine göre ise tanı yaşı, tanıda LDH düzeyi ve kök hücre naklinin sağkalımı artırdığı görülmüş olup istatistiksel olarak anlamlı olduğu saptanmıştır. Çalışmamızda, otolog kök hücre nakli yapılan hastalarda sağkalım oranının yüksek olduğu kliniklerden biri olduğumuz saptanmış olup, bu tedavinin özellikle yüksek risk Ewing sarkomu tedavisindeki önemi vurgulanmış oldu.
Purpose: Ewing sarcoma (ES) is a primary malignant tumor of the bone. Most cases are seen children and adolescents and prognosis is usually poor. Surgery, chemotherapy/autologeous stem cell transplantation and radiotherapy are used in the treatment of the disease. In this study, we aimed to examine the demographic and clinical data, treatment results and prognostic factors on survival of the patients with Ewing's sarcoma who treated and followed up in our clinic. Patients and Methods: Our retrospective study included 80 patients who were diagnosed with ES and admitted to the Pediatric Oncology Department of Faculty of Medicine of Ondokuz Mayıs University, between 1 January 2000 and 1 December 2021. In the evaluation of treatment results and prognostic factors, 71 patients included who was diagnosed and follow-up in only our clinic. Patients' age, gender, complaint, laboratory values, tumor location and volume, metastasis at the time of diagnosis, neoadjuvant treatment protocols, type of surgeries, response of tumor tissue to neoadjuvant treatment, adjuvant treatment protocols, recurrence and metastases in follow up, acute and late side effects of the treatments and the relationship of these data with survival were examined. The p values of <0.05 were considered as statistically significant. Results: The mean age at diagnosis of our cases was 14.1±7.6, male/female ratio was 1.2; the most common complaints were swelling (74.6%) and pain (69%). Extremity localization was common (52%) and the most common bone localization was the femur (18.3%) among all cases. Metastatic involvement was defined at the time of diagnosis in 29.5% of the patients. At the time of admission, LDH levels above 500 IU/L were seen in 27.5% and tumor volume more than 100 ml in 30% of the patients. Neoadjuvant chemotherapy protocols were given to 64.7% of the cases and surgery was done in 84.5% of the patients. Tumor resection constituted most of the surgical treatments. Autologous stem cell therapy was used to treatment in 26.7% of the cases. Various acute side effects observed in all patients due to the treatments, and late side effects were observed in 45% of them. In whole, 50.0% of the patients have died, while 40% continue their lives without disease. On other hand, %7,5 of patients continues their treatment and we could not reach the latest data in 2.5% of the patients. Conclusions and Recommendations: In our study; the age of admission was 10 years or older at the time of diagnosis, LDH value over 500 IU/L, histopathological response of tumor tissue to neoadjuvant chemotheraphy, not performing autologous stem cell transplantation, 100 ml or more tumor volume and use of incisional biopsy at the diagnosis has been shown significantly reduces survival (p<0,05). According to multivariate analysis, age at diagnosis, LDH level at diagnosis, and stem cell transplantation were found to increase survival and were found to be statistically significant. In our study, it was determined that we are one of the clinics with a high survival rate in patients who underwent autologous stem cell transplantation, and the importance of this treatment especially in the treatment of high-risk Ewing sarcoma was emphasized.
Purpose: Ewing sarcoma (ES) is a primary malignant tumor of the bone. Most cases are seen children and adolescents and prognosis is usually poor. Surgery, chemotherapy/autologeous stem cell transplantation and radiotherapy are used in the treatment of the disease. In this study, we aimed to examine the demographic and clinical data, treatment results and prognostic factors on survival of the patients with Ewing's sarcoma who treated and followed up in our clinic. Patients and Methods: Our retrospective study included 80 patients who were diagnosed with ES and admitted to the Pediatric Oncology Department of Faculty of Medicine of Ondokuz Mayıs University, between 1 January 2000 and 1 December 2021. In the evaluation of treatment results and prognostic factors, 71 patients included who was diagnosed and follow-up in only our clinic. Patients' age, gender, complaint, laboratory values, tumor location and volume, metastasis at the time of diagnosis, neoadjuvant treatment protocols, type of surgeries, response of tumor tissue to neoadjuvant treatment, adjuvant treatment protocols, recurrence and metastases in follow up, acute and late side effects of the treatments and the relationship of these data with survival were examined. The p values of <0.05 were considered as statistically significant. Results: The mean age at diagnosis of our cases was 14.1±7.6, male/female ratio was 1.2; the most common complaints were swelling (74.6%) and pain (69%). Extremity localization was common (52%) and the most common bone localization was the femur (18.3%) among all cases. Metastatic involvement was defined at the time of diagnosis in 29.5% of the patients. At the time of admission, LDH levels above 500 IU/L were seen in 27.5% and tumor volume more than 100 ml in 30% of the patients. Neoadjuvant chemotherapy protocols were given to 64.7% of the cases and surgery was done in 84.5% of the patients. Tumor resection constituted most of the surgical treatments. Autologous stem cell therapy was used to treatment in 26.7% of the cases. Various acute side effects observed in all patients due to the treatments, and late side effects were observed in 45% of them. In whole, 50.0% of the patients have died, while 40% continue their lives without disease. On other hand, %7,5 of patients continues their treatment and we could not reach the latest data in 2.5% of the patients. Conclusions and Recommendations: In our study; the age of admission was 10 years or older at the time of diagnosis, LDH value over 500 IU/L, histopathological response of tumor tissue to neoadjuvant chemotheraphy, not performing autologous stem cell transplantation, 100 ml or more tumor volume and use of incisional biopsy at the diagnosis has been shown significantly reduces survival (p<0,05). According to multivariate analysis, age at diagnosis, LDH level at diagnosis, and stem cell transplantation were found to increase survival and were found to be statistically significant. In our study, it was determined that we are one of the clinics with a high survival rate in patients who underwent autologous stem cell transplantation, and the importance of this treatment especially in the treatment of high-risk Ewing sarcoma was emphasized.
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