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Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi

Year 2010, Volume: 27 Issue: 2, 66 - 72, 10.02.2011

Abstract

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 % .

References

  • Ağaoğlu, F.Y., Kebudi, R., Almaç, Z., 2010. Pediatrik Ewing Sarkomalarda Lokal Tedavi:İstanbul Üniversitesi Onkoloji Enstitüsü Deneyimi. XVI.TPOG Ulusal Pediatrik Kanser Kongresi, Samsun. Kongre Kitabı. 119.
  • Bacci, G., Picci, P., Ferrari, S., 1998. Neoadjuvant chemotherapy for Ewing’s sarcoma of bone: no benefit observed after adding ifosfamide and etoposide to vincristine, actinomycin, cyclophosphamide, and doxorubicin in the maintenance phase-results of two sequential studies. Cancer. 82, 1174–1183.
  • Bernstein, M., Kovar, H., Paulussen, M., Randall, R.L., 2006. Ewing’s Sarcoma Family of Tumors: Ewing sarcoma of bone and soft tissue and the peripheral primitive neuroectodermal tumors. “Principles and Practice of Pediatric Onco-logy”(Ed.PA Pizzo, DG Poplack)’de, 5.baski, Lippincott Williams & Wilkins Philadelphia.1002-1032.
  • Bürgert, E.O., Nesbit, M.E., Ganisey, L.A., 1990. Multimodal therapy for the management of nonpelvie. localized Ewing’s sarcoma of bone: Intergroup study ffiSS-H. J. Clin. Oncol. 8, 1514-1524.
  • Cardenas-Cardos, R., Rivera-Luna, R., Lopez-Facundo, N.A., 1999. Ewing’s sarcoma: prognosis and survival in Mexican children from a single institution. Pediatr. Hematol. Oncol. 16, 519–523.
  • Cotterill, S.J., Ahrens, S., Paulussen, M., 2000. Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma Study Group. J. Clin. Oncol. 18, 3108-3114.
  • Ewing, J., 1921. Diffuse endothelioma of bone. Proc. NY Pathol. Soc. 21, 17-24.
  • Fizazi, K., Dohollou, N., Blay, J.Y., 1998. Ewing’s family of tumors in adults: multivariate analysis of survival and longterm results of multimodality therapy in 182 patients. J. Clin. Oncol.16, 3736-3743.
  • Göbel, V., Jürgens, H., Etspüler, G., 1987. Prognostic significance of tumor volume in localized Ewing’s sarcoma of bone in children and adolescents. J. Cancer Oncol. 113, 187-191. Güler, E., Çevik, S., Karakurum, G., 2010.
  • Ewing Sarkom Olgularımızın Değerlendirilmesi:Gaziantep Tıp Fakültesi Deneyimi. XVI.TPOG Ulusal Pediatrik Kanser Kongresi,Samsun. Kongre Kitabı 143.
  • Jenkin, R.D., Al-Fawaz, I., Al-Shabanah, M.O., 2002. Localized Ewing sarcoma/PNET of bone: prognostic factors and international data comparison. Med. Pediatr. Oncol. 39, 586–593.
  • Jürgens, H., Barrett, A., Dockhorn-Dworniczak, B., Winkelmann, 1998. Ewing sarcoma, in Cancer in Children: Clinical Management,Voute PA, Kalifa C and Barrett A, eds, 4th ed, Oxford Med. Publicat. 232-258.
  • Kantar, M., Keleş, Y.E., Arun, S., 2010. Ewing Ailesi Tümörleri Tedavi Sonuçları:Ege Üniversitesi Deneyimi. XVI. TPOG Ulusal Pediatrik Kanser Kongresi, Samsun, Kongre Kitabı 142-143.
  • Kennedy, J.G., Fielinghuysen, P., Hoang, B.H., 2003. Ewing sarcoma: curren; concepts in diagnosis and treatment, Curr. Opin. Pediatr. 1553-1557.
  • Khoury, J.D., 2005. Ewing sarcoma family of tumors. Adv. Anat. Pathol. 12, 212-220.
  • Kısmet E,Köseoğlu V, 2004.Turkiye Klinikleri J. Pediatr. Sp. Iss. 2, 899-903.
  • Kutluk, M.T., Yalcin, B., Akyüz, C., 2004. Treatment results and prognostic factors in Ewing Sarcoma. Pediatr. Hematol. Oncol. 21, 597-610.
  • Kutluk, T.,Yeşilipek, A., 2010.Türk Pediatrik Onkoloji Grubu/Türk Pediatrik Hematoloji Derneği Pediatrik Tümör ,2002-2009 Ulusal Pediatrik Kanser Kongresi, Samsun.
  • Nesbit, M.E., Gehan, E.A., Burgen, E.O., 1990. 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. 8,1664-1674.
  • Özkan A, Celkan T, Apak H, et al., 2005. Cerrahpaşa Tıp Fakültesi Ewing Sarkom Deneyimi:1983-2003. Ewing sarcoma: results of a single institute from Turkey,1983-2003.Türk Pediatri Arşivi. 40, 232-234.
  • Paulussen, M., Ahrens, S., Burdach, S., 1998. Primary metastatic (stage IV) Ewing tumor: survival analysisof 171 patients from the EICESS studies. European Intergroup Cooperative Ewing Sarcoma Studies. Ann Oncol. 9, 275–281.
  • Paulussen, M., Ahrens, S., Dunst, J., 2001.Localized Ewing Tumor of Bone:Final Results of the Cooperative Ewing’s Sarcoma Study CESS 86. J. Clin. Oncol.19, 1818-1829.
  • Redner, A., 2005. Malignant bone tumors. Pediatric Hematology and Oncology, Lanzkowsky P, ed, 4rd ed. California: Academic Press. 585-603.
  • Rodríguez-Galindo, C., Liu, T., Krasin, M.J., 2007. Analysis of prognostic factors in ewing sarcoma family of tumors: review of St. Jude Children’s Research Hospital studies. Cancer. 15, 375-384.
  • Sandoval, C., Meyer, W.H., Parham, D.M., 1996. Outcome in 43 children presenting with metastatic Ewing’s sarcoma: the St. Jude Children’s Research Hospital experience, 1962 to 1992. Med. Pediatr. Oncol. 26, 180–185.
  • Sarı, N., Toğral, G., Çetindağ, M.F., 2010. Treatment Result Of the Ewing Sarcoma of Bone and Prognostic Factors. Pediatric Blood Cancer. 54, 19-24.
  • Sevinir, B., Demirkaya, M., Yalçınkaya, Ü., 2010. Ewing Sarkomunda Klinik Özellikler ve Tedavi Sonuçları. XVI. TPOG Ulusal Pediatrik Kanser Kongresi, Samsun, Kongre Kitabı 141.
  • Villarroel, M., Tordecilla, J., Salgado, C., 1997. Multimodal therapy for children and adolescents with Ewing sarcoma: results of the First National Chilean Trial (1986–1991). Med. Pediatr. Oncol. 29, 190–196.
  • Wagner, L.V., Neel, M.D., Pappo, A.S., 2001. Fractures in pediatric Ewing sarcoma. J. Pediatr. Hematol. Oncol. 23, 568-571.
  • Widhe, B.,Widhe, T., 2000. Initial symptoms and clinical features in osteosarcoma and Ewing sarcoma. J. Bone Joint Surg. Am. 82, 667-674.
  • Yağcı, B., Kutluk, T., 2009. Ewing sarkom. Pediatr. Onkoloji. 995-1000.
Year 2010, Volume: 27 Issue: 2, 66 - 72, 10.02.2011

Abstract

References

  • Ağaoğlu, F.Y., Kebudi, R., Almaç, Z., 2010. Pediatrik Ewing Sarkomalarda Lokal Tedavi:İstanbul Üniversitesi Onkoloji Enstitüsü Deneyimi. XVI.TPOG Ulusal Pediatrik Kanser Kongresi, Samsun. Kongre Kitabı. 119.
  • Bacci, G., Picci, P., Ferrari, S., 1998. Neoadjuvant chemotherapy for Ewing’s sarcoma of bone: no benefit observed after adding ifosfamide and etoposide to vincristine, actinomycin, cyclophosphamide, and doxorubicin in the maintenance phase-results of two sequential studies. Cancer. 82, 1174–1183.
  • Bernstein, M., Kovar, H., Paulussen, M., Randall, R.L., 2006. Ewing’s Sarcoma Family of Tumors: Ewing sarcoma of bone and soft tissue and the peripheral primitive neuroectodermal tumors. “Principles and Practice of Pediatric Onco-logy”(Ed.PA Pizzo, DG Poplack)’de, 5.baski, Lippincott Williams & Wilkins Philadelphia.1002-1032.
  • Bürgert, E.O., Nesbit, M.E., Ganisey, L.A., 1990. Multimodal therapy for the management of nonpelvie. localized Ewing’s sarcoma of bone: Intergroup study ffiSS-H. J. Clin. Oncol. 8, 1514-1524.
  • Cardenas-Cardos, R., Rivera-Luna, R., Lopez-Facundo, N.A., 1999. Ewing’s sarcoma: prognosis and survival in Mexican children from a single institution. Pediatr. Hematol. Oncol. 16, 519–523.
  • Cotterill, S.J., Ahrens, S., Paulussen, M., 2000. Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma Study Group. J. Clin. Oncol. 18, 3108-3114.
  • Ewing, J., 1921. Diffuse endothelioma of bone. Proc. NY Pathol. Soc. 21, 17-24.
  • Fizazi, K., Dohollou, N., Blay, J.Y., 1998. Ewing’s family of tumors in adults: multivariate analysis of survival and longterm results of multimodality therapy in 182 patients. J. Clin. Oncol.16, 3736-3743.
  • Göbel, V., Jürgens, H., Etspüler, G., 1987. Prognostic significance of tumor volume in localized Ewing’s sarcoma of bone in children and adolescents. J. Cancer Oncol. 113, 187-191. Güler, E., Çevik, S., Karakurum, G., 2010.
  • Ewing Sarkom Olgularımızın Değerlendirilmesi:Gaziantep Tıp Fakültesi Deneyimi. XVI.TPOG Ulusal Pediatrik Kanser Kongresi,Samsun. Kongre Kitabı 143.
  • Jenkin, R.D., Al-Fawaz, I., Al-Shabanah, M.O., 2002. Localized Ewing sarcoma/PNET of bone: prognostic factors and international data comparison. Med. Pediatr. Oncol. 39, 586–593.
  • Jürgens, H., Barrett, A., Dockhorn-Dworniczak, B., Winkelmann, 1998. Ewing sarcoma, in Cancer in Children: Clinical Management,Voute PA, Kalifa C and Barrett A, eds, 4th ed, Oxford Med. Publicat. 232-258.
  • Kantar, M., Keleş, Y.E., Arun, S., 2010. Ewing Ailesi Tümörleri Tedavi Sonuçları:Ege Üniversitesi Deneyimi. XVI. TPOG Ulusal Pediatrik Kanser Kongresi, Samsun, Kongre Kitabı 142-143.
  • Kennedy, J.G., Fielinghuysen, P., Hoang, B.H., 2003. Ewing sarcoma: curren; concepts in diagnosis and treatment, Curr. Opin. Pediatr. 1553-1557.
  • Khoury, J.D., 2005. Ewing sarcoma family of tumors. Adv. Anat. Pathol. 12, 212-220.
  • Kısmet E,Köseoğlu V, 2004.Turkiye Klinikleri J. Pediatr. Sp. Iss. 2, 899-903.
  • Kutluk, M.T., Yalcin, B., Akyüz, C., 2004. Treatment results and prognostic factors in Ewing Sarcoma. Pediatr. Hematol. Oncol. 21, 597-610.
  • Kutluk, T.,Yeşilipek, A., 2010.Türk Pediatrik Onkoloji Grubu/Türk Pediatrik Hematoloji Derneği Pediatrik Tümör ,2002-2009 Ulusal Pediatrik Kanser Kongresi, Samsun.
  • Nesbit, M.E., Gehan, E.A., Burgen, E.O., 1990. 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. 8,1664-1674.
  • Özkan A, Celkan T, Apak H, et al., 2005. Cerrahpaşa Tıp Fakültesi Ewing Sarkom Deneyimi:1983-2003. Ewing sarcoma: results of a single institute from Turkey,1983-2003.Türk Pediatri Arşivi. 40, 232-234.
  • Paulussen, M., Ahrens, S., Burdach, S., 1998. Primary metastatic (stage IV) Ewing tumor: survival analysisof 171 patients from the EICESS studies. European Intergroup Cooperative Ewing Sarcoma Studies. Ann Oncol. 9, 275–281.
  • Paulussen, M., Ahrens, S., Dunst, J., 2001.Localized Ewing Tumor of Bone:Final Results of the Cooperative Ewing’s Sarcoma Study CESS 86. J. Clin. Oncol.19, 1818-1829.
  • Redner, A., 2005. Malignant bone tumors. Pediatric Hematology and Oncology, Lanzkowsky P, ed, 4rd ed. California: Academic Press. 585-603.
  • Rodríguez-Galindo, C., Liu, T., Krasin, M.J., 2007. Analysis of prognostic factors in ewing sarcoma family of tumors: review of St. Jude Children’s Research Hospital studies. Cancer. 15, 375-384.
  • Sandoval, C., Meyer, W.H., Parham, D.M., 1996. Outcome in 43 children presenting with metastatic Ewing’s sarcoma: the St. Jude Children’s Research Hospital experience, 1962 to 1992. Med. Pediatr. Oncol. 26, 180–185.
  • Sarı, N., Toğral, G., Çetindağ, M.F., 2010. Treatment Result Of the Ewing Sarcoma of Bone and Prognostic Factors. Pediatric Blood Cancer. 54, 19-24.
  • Sevinir, B., Demirkaya, M., Yalçınkaya, Ü., 2010. Ewing Sarkomunda Klinik Özellikler ve Tedavi Sonuçları. XVI. TPOG Ulusal Pediatrik Kanser Kongresi, Samsun, Kongre Kitabı 141.
  • Villarroel, M., Tordecilla, J., Salgado, C., 1997. Multimodal therapy for children and adolescents with Ewing sarcoma: results of the First National Chilean Trial (1986–1991). Med. Pediatr. Oncol. 29, 190–196.
  • Wagner, L.V., Neel, M.D., Pappo, A.S., 2001. Fractures in pediatric Ewing sarcoma. J. Pediatr. Hematol. Oncol. 23, 568-571.
  • Widhe, B.,Widhe, T., 2000. Initial symptoms and clinical features in osteosarcoma and Ewing sarcoma. J. Bone Joint Surg. Am. 82, 667-674.
  • Yağcı, B., Kutluk, T., 2009. Ewing sarkom. Pediatr. Onkoloji. 995-1000.
There are 31 citations in total.

Details

Primary Language English
Journal Section Internal Medical Sciences
Authors

Beyhan Bülbül

Murat Elli This is me

Ayhan Dağdemir This is me

Nevzat Dabak This is me

Sabri Acar This is me

Sancar Barış This is me

M.bekir Selçuk This is me

Gönül Oğur This is me

Publication Date February 10, 2011
Submission Date August 2, 2010
Published in Issue Year 2010 Volume: 27 Issue: 2

Cite

APA Bülbül, B., Elli, M., Dağdemir, A., Dabak, N., et al. (2011). Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi. Journal of Experimental and Clinical Medicine, 27(2), 66-72. https://doi.org/10.5835/jecm.v27i2.1393
AMA Bülbül B, Elli M, Dağdemir A, Dabak N, Acar S, Barış S, Selçuk M, Oğur G. Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi. J. Exp. Clin. Med. January 2011;27(2):66-72. doi:10.5835/jecm.v27i2.1393
Chicago Bülbül, Beyhan, Murat Elli, Ayhan Dağdemir, Nevzat Dabak, Sabri Acar, Sancar Barış, M.bekir Selçuk, and Gönül Oğur. “Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi”. Journal of Experimental and Clinical Medicine 27, no. 2 (January 2011): 66-72. https://doi.org/10.5835/jecm.v27i2.1393.
EndNote Bülbül B, Elli M, Dağdemir A, Dabak N, Acar S, Barış S, Selçuk M, Oğur G (January 1, 2011) Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi. Journal of Experimental and Clinical Medicine 27 2 66–72.
IEEE B. Bülbül, M. Elli, A. Dağdemir, N. Dabak, S. Acar, S. Barış, M. Selçuk, and G. Oğur, “Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi”, J. Exp. Clin. Med., vol. 27, no. 2, pp. 66–72, 2011, doi: 10.5835/jecm.v27i2.1393.
ISNAD Bülbül, Beyhan et al. “Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi”. Journal of Experimental and Clinical Medicine 27/2 (January 2011), 66-72. https://doi.org/10.5835/jecm.v27i2.1393.
JAMA Bülbül B, Elli M, Dağdemir A, Dabak N, Acar S, Barış S, Selçuk M, Oğur G. Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi. J. Exp. Clin. Med. 2011;27:66–72.
MLA Bülbül, Beyhan et al. “Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi”. Journal of Experimental and Clinical Medicine, vol. 27, no. 2, 2011, pp. 66-72, doi:10.5835/jecm.v27i2.1393.
Vancouver Bülbül B, Elli M, Dağdemir A, Dabak N, Acar S, Barış S, Selçuk M, Oğur G. Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi. J. Exp. Clin. Med. 2011;27(2):66-72.