Ewing's Sarcoma (Sarcoma, Ewing)

A malignant tumor of the bone which always arises in the medullary tissue, occurring more often in cylindrical bones. There are conspicuous foci of necrosis in association with irregular masses of small, regular, rounded or ovoid cells with very scanty cytoplasm. The tumor occurs usually before the age of 20, about twice as frequently in males as in females. (From Dorland, 27th ed; Stedman, 25th ed)

Also Known As:

Networked: 1407 relevant articles (73 outcomes, 145 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Sarcoma (Soft Tissue Sarcoma)
3.	Osteosarcoma (Osteogenic Sarcoma)
4.	Rhabdomyosarcoma
5.	Neoplasm Metastasis (Metastasis)

Experts

1.	Kleinerman, Eugenie S: 20 articles (02/2014 - 05/2002)
2.	Scotlandi, Katia: 20 articles (09/2012 - 03/2002)
3.	Picci, Piero: 19 articles (09/2012 - 03/2002)
4.	Zhou, Zhichao: 15 articles (02/2014 - 05/2002)
5.	Kovar, Heinrich: 15 articles (08/2012 - 08/2002)
6.	Lessnick, Stephen L: 14 articles (01/2013 - 05/2002)
7.	Manara, Maria Cristina: 13 articles (09/2012 - 03/2002)
8.	Serra, Massimo: 13 articles (04/2012 - 03/2002)
9.	Notario, Vicente: 11 articles (09/2013 - 04/2002)
10.	Ferrari, S: 11 articles (07/2011 - 01/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Ewing's Sarcoma:

1.	IfosfamideFDA LinkGeneric 12/01/1998 - "The present study suggests the importance of early use of ifosfamide in the treatment of patients with non metastatic Ewing's sarcoma of the extremity." 09/01/1992 - "No benefit of ifosfamide in Ewing's sarcoma: a nonrandomized study of the French Society of Pediatric Oncology." 09/01/2012 - "Mice implanted with Ewing's sarcoma tumours received the following treatments: saline, ifosfamide, ifosfamide + NAC concurrently, pre-treatment with NAC + ifosfamide, or NAC alone. " 07/01/2011 - "We report 3 adolescent patients who all recovered from their initial treatment for Ewing's sarcoma but developed a tubulopathy attributed to ifosfamide therapy. " 07/01/2011 - "Karyomegalic-like nephropathy, Ewing's sarcoma and ifosfamide therapy."
2.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 01/01/1982 - "A 5-year survival rose to 40.8% in 45 cases of Ewing's sarcoma, following adjuvant therapy with sarcolysin or cyclophosphamide administered after a course of radiation therapy in the dose of 50-60 Gy." 07/01/1981 - "It is concluded that the addition of cyclophosphamide alone is not adequate in the management of Ewing's sarcoma. " 06/01/1967 - "Cyclophosphamide in the management of Ewing's sarcoma." 10/01/1962 - "Cyclophosphamide therapy in children with Ewing's sarcoma." 06/01/2014 - "Cyclophosphamide-hydroxycamptothecin as second-line chemotherapy for advanced Ewing's sarcoma: experience of a single institution."
3.	Etoposide (VP 16)FDA LinkGeneric 05/01/2002 - "Adenovirus-E1A gene therapy enhances the in vivo sensitivity of Ewing's sarcoma to VP-16." 02/01/1989 - "In Ewing's sarcoma, the above combination with etoposide has resulted in high response rates." 11/01/2001 - "The cumulative second cancer risk five years after diagnosis of the Ewing tumor was 0.0093 for the total group, zero for patients without etoposide, and 0.0118 with etoposide. " 07/17/1998 - "EWS, a human protein involved in cellular transformation in Ewing's sarcoma tumors, contains an NH2-terminal transcriptional activation domain (NTD) which resembles that of SSAP in both amino acid composition and the ability to drive transcription to levels higher than VP16 in most cell types. " 10/01/1995 - "Calla positive acute lymphoblastic leukemia after etoposide-based therapy for Ewing's sarcoma."
4.	Doxorubicin (Adriamycin)FDA LinkGeneric 01/01/2006 - "Targeting CD99 in association with doxorubicin: an effective combined treatment for Ewing's sarcoma." 08/01/2001 - "In our own model of Ewing's sarcoma (ES), we have extensively investigated the involvement of the FasL/Fas pathway in doxorubicin (Dox)-induced apoptosis. " 10/06/1999 - "Treatment with an MMPI sensitized the Ewing's sarcoma cells to Fas-activating antibody and to doxorubicin-induced apoptosis. " 04/01/1998 - "The third patient was treated with IAC Adriamycin for Ewing's sarcoma of the right femur. " 05/01/1991 - "The impact of doxorubicin dose intensity on survival of patients with Ewing's sarcoma."
5.	Vincristine (Oncovin)FDA LinkGeneric 11/01/2001 - "Six hundred ninety Ewing tumor patients were treated between 1992 and 1999 with local therapy and vincristine. " 04/01/1996 - "A 16-year-old male suffering from Ewing's sarcoma of the pelvis was treated with vincristine as part of his chemotherapeutic protocol. " 01/01/2011 - "In vivo studies confirmed the cytostatic efficay of NBDHEX and its positive interaction with vincristine in Ewing's sarcoma cells, and also indicated a positive effect against the metastatisation of osteosarcoma cells. " 08/01/1995 - "A patient with Ewing's sarcoma and demyelinating type Charcot-Marie-Tooth disease (CMT) developed severe neuropathy after receiving a total vincristine dose of 6 mg. Recovery was slow and incomplete. " 03/01/1976 - "Ewing's sarcoma: adjuvant total body irradiation, cyclophosphamide and vincristine."
6.	Dactinomycin (Ac-De)FDA LinkGeneric 04/01/1970 - "Treatment of metastatic Ewing's sarcoma with actinomycin D (NSC-3053)." 10/05/2003 - "Actinomycin D is an anti-cancer drug commonly used in the treatment of paediatric malignancies such as Wilms' tumour, Ewing's sarcoma and rhabdomyosarcoma. " 01/01/2004 - "In this study, we showed that etoposide and actinomycin D kill Ewing tumor cells (RD-ES cell line) mainly via a caspase-dependent mechanism. " 10/16/1991 - "Increasing dactinomycin dose intensity was associated with a poorer outcome in treatment of osteogenic sarcoma and Ewing's sarcoma, most likely resulting from regimens with a higher dactinomycin dose intensity having a lower doxorubicin dose intensity. " 01/01/1998 - "A higher rate of good responses was achieved with the use of ifosfamide and dactinomycin in addition to a conventional three-drug VAC regimen, suggesting that these drugs should be included from the beginning in neoadjuvant regimens for the treatment of Ewing's sarcoma. "
7.	AnthracyclinesIBA 01/01/2014 - "For both Wilms' tumour and Ewing's sarcoma a significant difference in event-free and overall survival in favour of treatment with anthracyclines was identified, although for Wilms' tumour the significant difference in overall survival disappeared with long-term follow-up. " 01/01/2011 - "For both Wilms' tumour and Ewing's sarcoma a significant difference in event-free and overall survival in favour of treatment with anthracyclines was identified, although for Wilms' tumour the significant difference in overall survival disappears with long-term follow-up. " 01/01/2009 - "For both Wilms' tumour and Ewing's sarcoma a significant difference in survival in favour of treatment with anthracyclines was identified. " 01/01/2009 - "For Wilms' tumour, rhabdomyosarcoma/undifferentiated sarcoma, Ewing's sarcoma, and non-Hodgkin lymphoma only 1 RCT was available and therefore, no definitive conclusions can be made about the antitumour efficacy of treatment with or without anthracyclines in these tumours. " 01/01/2014 - "For Wilms' tumour, rhabdomyosarcoma and undifferentiated sarcoma, Ewing's sarcoma, non-Hodgkin lymphoma, hepatoblastoma and AML only one RCT was available for each type and, therefore, no definitive conclusions can be made about the antitumour efficacy of treatment with or without anthracyclines in these tumours. "
8.	Protons (Proton)IBA 11/01/2009 - "Efficacy of Proton Beam Therapy in the Treatment of Ewing's Sarcoma of the Paranasal Sinuses and Anterior Skull Base." 03/01/2012 - "Proton radiotherapy for pediatric Ewing's sarcoma: initial clinical outcomes." 07/01/2009 - "Treatment included pre- and/or postoperative photon/proton XRT with or without radical resection; patients with osteosarcoma and Ewing's sarcoma received chemotherapy. " 01/01/2014 - "Proton radiation has been used safely and effectively for medulloblastoma, primitive neuro-ectodermal tumors, craniopharyngioma, ependymoma, germ cell intracranial tumors, low-grade glioma, retinoblastoma, rhabdomyosarcoma and other soft tissue sarcomas, Ewing's sarcoma and other bone sarcomas. " 11/01/2006 - "Options for difficult Ewing's sarcoma and osteosarcoma situations and multimodality solutions, including 1 mCi/kg of samarium and proton therapy, are discussed. "
9.	Dihydrotachysterol (AT 10)IBA 01/01/2002 - "We report a 32-year-old women with a pelvic Ewing's sarcoma, who developed skeletal metastases within 20 months of diagnosis but following treatment remains disease-free at 10 years. " 04/01/2015 - "Patients with Ewing's sarcoma have decreased cause-specific survival at 10 years when metastatic at presentation, axial tumor location, tumor size>10cm, and patient age≥20 years." 02/01/1995 - "Important advances discussed over this period include: establishment of sarcoma teams in major oncology centers; staging systems for both soft tissue and osseous sarcomas; demonstration of genetic determinants in the development of, at least, some of the sarcomas; the revolutionary change in quality of diagnostic imaging by the introduction of CT and MRI; use of immunohistochemistry in diagnostic pathology; the drastic gains in survival of patients with osteogenic sarcoma, Ewing's sarcoma and rhabdomyosarcoma due to the efficacy of multi-drug and multi-cycle chemotherapy protocols; major advances in surgical techniques which have made limb salvage practical; cell lines derived from human sarcomas have been shown to have in vitro radiation sensitivity comparable to that of cell lines from epithelial tumors; the combination of conservative surgery and moderate doses of radiation yields local control and survival results equivalent to that of radical surgery with a much improved functional and cosmetic outcome; intra-operative electron beam radiation therapy improves the outcome of patients with retroperitoneal sarcomas when given after grossly complete resection combined with external beam radiation therapy (pre- or postoperatively); radiation is a highly effective alternative to extensive surgery for desmoid tumors; local control of giant cell tumors by modern radiation techniques is approximately 80% and the incidence of radiation induced tumors at 10 years is approximately 3%; to decrease the incidence of radiation induced sarcoma, resection has replaced radiation in the management of selected patients with primary Ewing's sarcoma when the response to chemotherapy has been excellent and the morbidity/functional decrement consequent upon the surgery judged reasonable; proton beam radiation therapy has been accepted as being superior to conventional external beam radiation therapy for chondrosarcoma and chordoma of the skull base; and attempts to utilize brachytherapy for sarcomas of the spine/sacrum appear to offer promise. "
10.	Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA 06/04/2007 - "These results indicate that TAMs in Ewing's sarcoma are capable of osteoclast differentiation by both RANKL-dependent and TNF-alpha-dependent mechanisms and that Ewing's sarcoma cells produce osteoclastogenic factor(s). " 06/01/2007 - "Ewing's sarcoma cells are highly susceptible to apoptosis via tumor necrosis factor apoptosis-inducing ligand (TRAIL). " 02/22/2001 - "Cytotoxicity of Tumor necrosis factor related apoptosis-inducing ligand towards Ewing's sarcoma cell lines." 02/01/1999 - "Monomorphic HLA class I-(non-A, non-B) expression on Ewing's tumor cell lines, modulation by TNF-alpha and IFN-gamma." 08/01/1997 - "We examined the influence of ionizing radiation on TNF alpha production in a human Ewing's sarcoma cell line in vitro and in vivo. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 02/01/2010 - "Progress in the treatment of Ewing's sarcoma, the second most common bone tumour in children and adolescents, has improved survival from about 10% in the period before chemotherapy was introduced to about 75% today for patients with localised tumours. " 09/01/2001 - "The most effective treatment of Ewing's sarcoma is chemotherapy." 08/01/1998 - "As most failures in Ewing's sarcoma are systemic, improved EFS requires more effective systemic chemotherapy. " 01/01/2015 - "After undergoing chemotherapy and a complete surgical resection of the tumour, the patient remains disease-free 10 years later, indicating that the biology of Ewing's sarcoma may be more important than time to diagnosis in determining outcome. " 04/01/1981 - "The study indicated that intensive chemotherapy and RT significantly improved the local control and survival of patients with localized Ewing's sarcoma. "
2.	Radiotherapy 07/01/2003 - "The aim of the study was to evaluate the oncologic results of combined surgery and radiotherapy of pelvic Ewing's sarcoma. " 08/01/1998 - "A multidisciplinary study investigating radiotherapy in Ewing's sarcoma: end results of POG #8346. " 07/01/2014 - "Expecting contaminated (R1) margins in patients with Ewing's sarcoma (e.g., in critical locations such as the pelvis), radiotherapy only is better than hoping for the "cure" of insufficient resections margins with a combination of both methods. " 04/01/2013 - "Thus, dose escalation in the radiotherapy of pelvic Ewing's sarcoma can be more easily achieved using IMRT." 11/01/2012 - "Radiotherapy constitutes an essential element in the multimodal therapy of Ewing's sarcoma. "
3.	Adjuvant Chemotherapy 04/15/1986 - "These results are compared with the results of 68 patients with Ewing's sarcoma who were treated during the same period with radiation therapy and adjuvant chemotherapy and reported in a previous study. " 01/01/1982 - "The results at 5 years of an adjuvant chemotherapy trial in primary Ewing's sarcoma, started in 1973, are presented. " 04/01/1981 - "We concluded that further studies, in which stratification for site of primary disease and LDH levels would be used, are necessary to clarify the role of intensive adjuvant chemotherapy in Ewing's sarcoma." 05/01/2011 - "It was decided to treat the lesion as for osseous Ewing's sarcoma with wide resection followed by systemic adjuvant chemotherapy. " 01/01/2000 - "Prognostic factors in nonmetastatic Ewing's sarcoma of bone treated with adjuvant chemotherapy: analysis of 359 patients at the Istituto Ortopedico Rizzoli."
4.	Heterologous Transplantation (Xenotransplantation) 10/31/2002 - "Since the possibility of using IFNs for Ewing tumor treatments may be of interest, we have evaluated the efficacy of Hu-IFNs in a nude mice model of Ewing tumor xenografts. " 02/01/2010 - "Using immunohistochemistry, we examined the expression pattern of DLL4 in 14 patient samples and two xenograft mouse models of Ewing's sarcoma. " 05/01/2004 - "Changed adhesion molecule profile of Ewing tumor cell lines and xenografts under the influence of ionizing radiation." 03/01/2002 - "Control experiments were performed using two Ewing's sarcoma xenograft models. " 01/01/2012 - "In addition, we show that STEAP1 knockdown reduces Ewing tumor proliferation, anchorage-independent colony formation as well as invasion in vitro and decreases growth and metastasis of Ewing tumor xenografts in vivo. "
5.	Aftercare (After-Treatment) 10/01/2008 - "We report two rare cases of very late local recurrence of Ewing's sarcoma, occurring 16 years and 19 years after treatment of the primary tumour. " 02/01/2008 - "Long-term clinical and functional outcomes after treatment for localized Ewing's tumor of the lower extremity." 07/01/1997 - "Our data indicate that the peripheral CD34 positive cell count prior to leukapheresis is the best parameter for predicting the efficacy of peripheral stem cell apheresis in Ewing Tumor patients after treatment with standardized chemotherapy." 09/01/1998 - "Second malignancies after treatment for Ewing's sarcoma: a report of the CESS-studies." 11/01/2008 - "We report a case of 12-year-old boy who after treatment for Ewing's sarcoma of proximal fibula subsequently presented, 4 years later, with a high-grade fibroblastic osteosarcoma of the proximal humerus. "