NCCN Guidelines Insights: Bone Cancer, Version 2.2017.

The NCCN Guidelines for Bone Cancer provide interdisciplinary recommendations for treating chordoma, chondrosarcoma, giant cell tumor of bone, Ewing sarcoma, and osteosarcoma. These NCCN Guidelines Insights summarize the NCCN Bone Cancer Panel's guideline recommendations for treating Ewing sarcoma. The data underlying these treatment recommendations are also discussed.

[1]  W. Winkelmann,et al.  Primary metastatic (stage IV) Ewing tumor: survival analysis of 171 patients from the EICESS studies. European Intergroup Cooperative Ewing Sarcoma Studies. , 1998, Annals of oncology : official journal of the European Society for Medical Oncology.

[2]  H. Kovar,et al.  Ewing's sarcoma family of tumors: current management. , 2006, The oncologist.

[3]  P. Leavey,et al.  Dose-intensified compared with standard chemotherapy for nonmetastatic Ewing sarcoma family of tumors: a Children's Oncology Group Study. , 2009, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[4]  S. Hopyan,et al.  Clinical outcome of children and adults with localized Ewing sarcoma , 2010, Cancer.

[5]  M. Link,et al.  Intensive therapy with growth factor support for patients with Ewing tumor metastatic at diagnosis: Pediatric Oncology Group/Children's Cancer Group Phase II Study 9457--a report from the Children's Oncology Group. , 2006, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[6]  M Beth McCarville,et al.  Combination of gemcitabine and docetaxel in the treatment of children and young adults with refractory bone sarcoma , 2008, Cancer.

[7]  W. Tap,et al.  The Multidisciplinary Management of Osteosarcoma , 2009, Current treatment options in oncology.

[8]  S. Burchill.  Molecular abnormalities in Ewing’s sarcoma , 2008, Expert review of anticancer therapy.

[9]  W. Winkelmann,et al.  Localized Ewing tumor of bone: final results of the cooperative Ewing's Sarcoma Study CESS 86. , 2001, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[10]  W. Winkelmann,et al.  Radiation therapy in Ewing's sarcoma: an update of the CESS 86 trial. , 1995, International journal of radiation oncology, biology, physics.

[11]  W. Winkelmann,et al.  Evaluation of prognostic factors in a tumor volume-adapted treatment strategy for localized Ewing sarcoma of bone: The CESS 86 experience , 1999 .

[12]  J. Zell,et al.  Analysis of prognostic factors in Ewing sarcoma using a population‐based cancer registry , 2010, Cancer.

[13]  U. Dirksen,et al.  The value of high‐dose chemotherapy in patients with first relapsed Ewing sarcoma , 2014, Pediatric blood & cancer.

[14]  T. Triche,et al.  Ifosfamide with mesna uroprotection and etoposide: an effective regimen in the treatment of recurrent sarcomas and other tumors of children and young adults. , 1987, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[15]  B. Wen,et al.  Dose response and local control using radiotherapy in non‐metastatic Ewing sarcoma , 2007, Pediatric blood & cancer.

[16]  S. Ferrari,et al.  Neoadjuvant chemotherapy for Ewing's sarcoma of bone , 1998, Cancer.

[17]  M. Bernstein,et al.  Cyclophosphamide plus topotecan in children with recurrent or refractory solid tumors: a Pediatric Oncology Group phase II study. , 2001, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[18]  P. Leavey,et al.  Prognostic factors for patients with Ewing sarcoma (EWS) at first recurrence following multi‐modality therapy: A report from the Children's Oncology Group , 2008, Pediatric blood & cancer.

[19]  M Beth McCarville,et al.  Temozolomide and intravenous irinotecan for treatment of advanced Ewing sarcoma , 2007, Pediatric blood & cancer.

[20]  C. Beauchamp.  Definitive radiotherapy for ewing tumors of extremities and pelvis: long-term disease control, limb function, and treatment toxicity , 2009 .

[21]  J. Habrand,et al.  No benefit of ifosfamide in Ewing's sarcoma: a nonrandomized study of the French Society of Pediatric Oncology. , 1992, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[22]  P. Picci,et al.  Italian Cooperative Study for the treatment of children and young adults with localized Ewing sarcoma of bone , 1999, Cancer.

[23]  T. Merchant,et al.  Survival after recurrence of Ewing Tumors , 2002, Cancer.

[24]  I. Helenius,et al.  Surgically treated patients with axial and peripheral Ewing's sarcoma family of tumours: A population based study in Finland during 1990-2009. , 2015, European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology.

[25]  S. Donaldson,et al.  Comparative evaluation of local control strategies in localized Ewing sarcoma of bone: A report from the Children's Oncology Group , 2015, Cancer.

[26]  S. Ferrari,et al.  Prognostic factors in non-metastatic Ewing's sarcoma tumor of bone: An analysis of 579 patients treated at a single institution with adjuvant or neoadjuvant chemotherapy between 1972 and 1998 , 2006, Acta oncologica.

[27]  A. Angiolillo,et al.  Ifosfamide, carboplatin, and etoposide (ICE) reinduction chemotherapy in a large cohort of children and adolescents with recurrent/refractory sarcoma: The Children's Cancer Group (CCG) experience , 2005, Pediatric blood & cancer.

[28]  T. Triche,et al.  The Ewing family of tumors--a subgroup of small-round-cell tumors defined by specific chimeric transcripts. , 1994, The New England journal of medicine.

[29]  C. Pratilas,et al.  Ifosfamide dose‐intensification for patients with metastatic Ewing sarcoma , 2015, Pediatric blood & cancer.

[30]  C. Denny.  Gene rearrangements in Ewing's sarcoma. , 1996, Cancer investigation.

[31]  E. Gehan,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. , 1990, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[32]  H. Jürgens,et al.  Topotecan and cyclophosphamide in patients with refractory or relapsed Ewing tumors , 2006, Pediatric blood & cancer.

[33]  S. Donaldson,et al.  Local control in pelvic Ewing sarcoma: analysis from INT-0091--a report from the Children's Oncology Group. , 2006, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[34]  P. Houghton,et al.  Phase I Trial of Temozolomide and Protracted Irinotecan in Pediatric Patients with Refractory Solid Tumors , 2004, Clinical Cancer Research.

[35]  A. Davidoff,et al.  Timing of Surgery and the Role of Adjuvant Radiotherapy in Ewing Sarcoma of the Chest Wall: A Single-institution Experience , 2012, Annals of Surgical Oncology.

[36]  L. Wexler,et al.  Irinotecan and temozolomide for Ewing sarcoma: The Memorial Sloan‐Kettering experience , 2009, Pediatric blood & cancer.

[37]  H. Jürgens,et al.  Prognostic significance of tumor volume in localized Ewing's sarcoma of bone in children and adolescents , 2004, Journal of Cancer Research and Clinical Oncology.

[38]  U. Dirksen,et al.  Management and Outcome of Ewing Sarcoma of the Head and Neck , 2016, Pediatric blood & cancer.

[39]  W. Winkelmann,et al.  Local therapy in localized Ewing tumors: results of 1058 patients treated in the CESS 81, CESS 86, and EICESS 92 trials. , 2003, International journal of radiation oncology, biology, physics.

[40]  N. Dubrawsky.  Cancer statistics , 1989, CA: a cancer journal for clinicians.

[41]  K. S. Hall,et al.  Post‐relapse survival in patients with Ewing sarcoma , 2015, Pediatric blood & cancer.

[42]  C. Rodríguez-Galindo,et al.  Vincristine, irinotecan, and temozolomide in patients with relapsed and refractory Ewing sarcoma , 2013, Pediatric blood & cancer.

[43]  A. Craft,et al.  Prognostic factors in Ewing's tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing's Sarcoma Study Group. , 2000, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[44]  D. Vanel,et al.  Treatment of nonmetastatic Ewing’s sarcoma family tumors of the spine and sacrum: the experience from a single institution , 2009, European Spine Journal.

[45]  W. Gerald,et al.  Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family. , 2000, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[46]  U. Dirksen,et al.  The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES) , 2010, Cancer.

[47]  W. Winkelmann,et al.  Results of the EICESS-92 Study: two randomized trials of Ewing's sarcoma treatment--cyclophosphamide compared with ifosfamide in standard-risk patients and assessment of benefit of etoposide added to standard treatment in high-risk patients. , 2008, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[48]  B. Bui,et al.  Prognostic factors in localized Ewing's tumours and peripheral neuroectodermal tumours: the third study of the French Society of Paediatric Oncology (EW88 study) , 2001, British Journal of Cancer.

[49]  F. Askin,et al.  Ewing's sarcoma of bone: clinicopathologic aspects of 303 cases from the Intergroup Ewing's Sarcoma Study. , 1983, Human pathology.

[50]  J. Blay,et al.  Etoposide and carbo‐or cisplatin combination therapy in refractory or relapsed Ewing sarcoma: A large retrospective study , 2015, Pediatric blood & cancer.

[51]  E. Gehan,et al.  Ewing's sarcoma metastatic at diagnosis results and comparisons of two intergroup Ewing's sarcoma studies , 1990, Cancer.

[52]  S. Spunt,et al.  Analysis of prognostic factors in ewing sarcoma family of tumors , 2007, Cancer.

[53]  K. Hess,et al.  Prognostic factors and patterns of relapse in ewing sarcoma patients treated with chemotherapy and r0 resection. , 2015, International journal of radiation oncology, biology, physics.

[54]  T. Merchant,et al.  Definitive irradiation in multidisciplinary management of localized Ewing sarcoma family of tumors in pediatric patients: outcome and prognostic factors. , 2004, International journal of radiation oncology, biology, physics.

[55]  S. Steinberg,et al.  Ifosfamide and etoposide plus vincristine, doxorubicin, and cyclophosphamide for newly diagnosed Ewing's sarcoma family of tumors , 1996, Cancer.

[56]  A. Huvos,et al.  Long-term event-free survival after intensive chemotherapy for Ewing's family of tumors in children and young adults. , 2003, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[57]  S. Ferrari,et al.  Therapy and survival after recurrence of Ewing's tumors: the Rizzoli experience in 195 patients treated with adjuvant and neoadjuvant chemotherapy from 1979 to 1997. , 2003, Annals of oncology : official journal of the European Society for Medical Oncology.

[58]  N. Tarbell,et al.  Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Chest Wall: Impact of Initial Versus Delayed Resection on Tumor Margins, Survival, and Use of Radiation Therapy , 2003, Annals of surgery.

[59]  W. Winkelmann,et al.  Evaluation of prognostic factors in a tumor volume-adapted treatment strategy for localized Ewing sarcoma of bone: the CESS 86 experience. Cooperative Ewing Sarcoma Study. , 1999, Medical and pediatric oncology.

[60]  U. Dirksen,et al.  Cyclophosphamide compared with ifosfamide in consolidation treatment of standard-risk Ewing sarcoma: results of the randomized noninferiority Euro-EWING99-R1 trial. , 2014, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[61]  W. Winkelmann,et al.  Radiotherapy in Ewing tumors of the vertebrae: treatment results and local relapse analysis of the CESS 81/86 and EICESS 92 trials. , 2005, International journal of radiation oncology, biology, physics.

[62]  S. Donaldson,et al.  Treatment of metastatic Ewing's sarcoma or primitive neuroectodermal tumor of bone: evaluation of combination ifosfamide and etoposide--a Children's Cancer Group and Pediatric Oncology Group study. , 2004, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[63]  E. Pignotti,et al.  Long-term outcome for patients with non-metastatic Ewing's sarcoma treated with adjuvant and neoadjuvant chemotherapies. 402 patients treated at Rizzoli between 1972 and 1992. , 2004, European journal of cancer.

[64]  E. Gehan,et al.  Multimodal therapy for the management of nonpelvic, localized Ewing's sarcoma of bone: intergroup study IESS-II. , 1990, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[65]  A. Lazar,et al.  Ewing’s Sarcoma: Standard and Experimental Treatment Options , 2009, Current treatment options in oncology.

[66]  S. Donaldson,et al.  Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. , 2003, The New England journal of medicine.

[67]  U. Dirksen,et al.  Risk of recurrence and survival after relapse in patients with Ewing sarcoma , 2011, Pediatric blood & cancer.

[68]  W. Winkelmann,et al.  Primary metastatic osteosarcoma: presentation and outcome of patients treated on neoadjuvant Cooperative Osteosarcoma Study Group protocols. , 2003, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[69]  T. Merchant,et al.  Definitive surgery and multiagent systemic therapy for patients with localized Ewing sarcoma family of tumors , 2005, Cancer.

[70]  J. Healey,et al.  Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: a report from the Children's Oncology Group. , 2012, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[71]  S. Wolden,et al.  Radiation for bone metastases in Ewing sarcoma and rhabdomyosarcoma , 2015, Pediatric blood & cancer.

[72]  P. Meyers,et al.  Pilot study of topotecan and high-dose cyclophosphamide for resistant pediatric solid tumors. , 2000, Medical and pediatric oncology.

[73]  R. Makuch,et al.  Determination of prognostic factors and their influence on therapeutic results in patients with Ewing's sarcoma , 1980, Cancer.