Çocukluk Çağı Lokal Yüksek Risk Ewing Sarkomu’nda Yüksek doz Kemoterapi ve Otolog Kök Hücre Nakli Ne Zaman Yapılmalı?: Olgu Sunumu

Yıl 2022, Cilt: 3 Sayı: 1, 31 - 35, 12.03.2022

Hatice Mine Çakmak , Yaşar Bildirici , Fuzuli Tuğrul , Meral Bahar Murat Baloğlu Müge Gündoğdu

https://doi.org/10.48176/esmj.2022.53

Öz

Lokalize yüksek risk Ewing sarkomu tanısı olan hastalarda tedavi protokolleri farklıdır. Euro-Ewing 2012 protokolünde sırasıyla indüksiyon kemoterapisi, lokal tedavi ve yüksek doz kemoterapi ve otolog kök hücre nakli yapılırken, COG (Children’s Oncology Group) protokolünde indüksiyon kemoterapisi, lokal tedavi ve sonrasında kemoterapi ile 14 küre tedavi tamamlanmaktadır. Bu hastalıkta standard kemoterapiye eklenecek biyolojik ajanlara ve idame tedavisine ihtiyaç vardır. Burada on beş yaşında, lokalize yüksek risk pelvik kaynaklı (tanıda tümör hacmi >400 ml), Ewing sarkomu tanılı olgumuz tartışıldı. COG kemoterapi protokolü başlandı. Üç kür sonrası denetlemede inoperabl olduğu için radyoterapi uygulandı. Dördüncü kürden sonra kök hücre toplandı. Kemoterapisinin 14 küre tamamlanması planlandı. Nüks olması halinde indüksiyondan sonra, yüksek doz kemoterapi ve otolog kök hücre nakli yapılması planlandı. Tanıda tümör hacmi 400 ml’nin üstünde tümör hacmi olan non-metastatik Ewing sarkomu, yüksek risklidir, ilk remisyonda cerrahi sonrası yüksek doz kemoterapi ve otolog kök hücre transplantasyonunun yapılmasının sağ kalımı arttırdığına dair makaleler mevcuttur. Ancak, rutin olarak uygulanması konusunda fikir birliğine varılamamıştır.

Anahtar Kelimeler

Anahtar kelimeler: Ewing sarkomu, çocuk, non-metastatik, yüksek risk, periferik kök hücre toplanması, otolog hematopoetik kök hücre nakli

When Should High Dose Chemotherapy and Autologous Stem Cell Transplantation Be Performed in a Pediatric Local High-Risk Ewing Sarcoma?: A Case Report

Öz

In local high-risk Ewing Sarcoma, treatment approaches differ between COG (Children’s Oncology Group) and Euro Ewing 2012 protocols. In Euro-Ewing protocol, high-dose chemotherapy and autologous stem cell transplantation is performed after local therapy. COG protocol includes fourteen chemotherapy regimens and local therapy after the fourth chemotherapy regimen. Targeted agents added to standard chemotherapy and novel agents for maintenance are warranted (11-14). Here, we discuss the time of local therapy in stem cell rescue, high-dose chemotherapy, and autologous stem cell transplantation in this aggressive tumor. Our fifteen-year-old patient had right pelvic Ewing sarcoma with a volume higher than 400 ml. After three chemotherapy regimens, the tumor was inoperable and radiotherapy was applied. Stem cell rescue is performed after the fourth chemotherapy regimen. High-dose chemotherapy and autologous stem cell transplantation are planned for relapse consolidation (17). There is no consensus about the indication of high-dose chemotherapy and autologous stem cell transplantation in high risc Ewing sarcoma with an initial tumor volume > 400 ml.

Anahtar Kelimeler

Keywords: Ewing Sarcoma, children, non-metastatic, high risk, stem cell rescue, autologous hematopoetic stem cell transplantation

Kaynakça

• 1) Jedlicka P. Ewing Sarcoma, an enigmatic malignancy of likely progenitor cell origin, driven by transcription factor oncogenic fusions. Int J Clin Exp Pathol. 2010;3(4):338. Epub 2010 Mar 19.
• 2) Nesbit ME Jr, Gehan EA, Burgert EO, et al. Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: a long-term follow-up of the First Intergroup study. J Clin Oncol. 1990;8(10):1664.
• 3) Sankar S, Lessnick SL. Prmiscuous partnerships in Ewing’s Sarcoma.Cancer genetics 2011;204:351-365.
• 4) Brasme JF, Chalumeau M, Oberlin O, et. al. Time to diagnosis of Ewing tumors in children and adolescents is not associated with metastasis or survival: a prospective multicenter study of 436 patients. J Clin Oncol 2014; 32:1935-1940.
• 5) Wilhe B, Widhe T. Initial symptoms and clinical feature in osteosarcoma and Ewing Sarcoma. J Bone Joint Surg Am 2000; 82:667-674.
• 6) Rimondi E, Rossi G, Bartalena T, et al. Percutaneus CT-guided biopsy of the musculoskeletal system: results of 2027 cases. Eur J Radiol 2011;77:34-42.
• 7) Schuck A, Ahrens S, Paulussen M. Local therapy in localized Ewing tumors: results of 1058 patients treated in the CESS 81, CESS 86, and EICESS 92 trials. Int J Radiat Oncol Biol Phys. 2003 1;55:168-77.
• 8) Gurney JG, Swensen AR, Bultreys M. Cancer incidence and survival among children and adolescents: United States SEER program 1975-1995. In: RRRRies LAG, Smith MA, Gurney JG, et.a. eds. Malignant tumors, Bethesda, MD: National Cancer Institute, 1999;99-110.
• 9) Ferrari S, Mercuri M, Rosito P, et al. Ifosfamide and actinomycin-D, added in the induction phase to vincristine, cyclophosphamide and doxorubicin, improve histologic response and prognosis in patients with non metastatic Ewing's sarcoma of the extremity. J Chemother. 1998;10(6):484.
• 10) Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. 2003;348:694.
• 11) Womer RB, West DC, Krailo MD, et al. Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: a report from the Children's Oncology Group. J Clin Oncol. 2012;30:4148-54.
• 12) Dirksen U, Brennan B, Le Deley MC, et al. Euro-E.W.I.N.G. 99 and Ewing 2008 Investigators. High-Dose Chemotherapy Compared With Standard Chemotherapy and Lung Radiation in Ewing Sarcoma With Pulmonary Metastases: Results of the European Ewing Tumour Working Initiative of National Groups, 99 Trial and EWING 2008. J Clin Oncol. 2019;37:3192-3202.
• 13) Gorlick R, Janeway K A, Adamson P C. Dose Intensification Improves the Outcome of Ewing Sarcoma. J Clin Oncol 2008 Sep 12:JCO2018793489. doi: 10.1200/JCO.2018.79.3489. Epub ahead of print. PMID: 30204546.
• 14) Brennan B, Kirton L, Marec-Berard P. Comparison of two chemotherapy regimens in Ewing sarcoma (ES): Overall and subgroup results of the Euro Ewing 2012 randomized trial. J Clin Oncol. 2020;38:11500-11500.
• 15) Puri A, Gulia A, Jambhekar NA, Laskar S. Results of surgical resection in pelvic Ewing's sarcoma. J Surg Oncol. 2012 Sep 15;106:417-22.
• 16 )Dunst J, Schuck A. Role of radiotherapy in Ewing tumors. Pediatr Blood Cancer. 2004 ;42:465-70.
• 17) Tenneti P, Zahid U, Iftikhar A, Yun S, Sohail A, Warraich Z, et al. Role of High-Dose Chemotherapy and Autologous Hematopoietic Cell Transplantation for Children and Young Adults with Relapsed Ewing's Sarcoma: A Systematic Review. Sarcoma. 2018; 3;2018:2021.