Ewing Sarcoma (Sarcoma, Ewing)

A malignant tumor of the bone which always arises in the medullary tissue, occurring more often in cylindrical bones. The tumor occurs usually before the age of 20, about twice as frequently in males as in females.

Also Known As:

Networked: 3743 relevant articles (223 outcomes, 466 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1.	Lessnick, Stephen L: 57 articles (01/2022 - 05/2002)
2.	Dirksen, Uta: 50 articles (01/2022 - 07/2006)
3.	Picci, Piero: 46 articles (06/2021 - 03/2002)
4.	Scotlandi, Katia: 45 articles (04/2022 - 03/2002)
5.	Kovar, Heinrich: 39 articles (01/2021 - 08/2002)
6.	Toretsky, Jeffrey A: 36 articles (03/2022 - 10/2004)
7.	Lawlor, Elizabeth R: 32 articles (10/2022 - 09/2008)
8.	Delattre, Olivier: 30 articles (05/2022 - 10/2002)
9.	Manara, Maria Cristina: 30 articles (04/2022 - 03/2002)
10.	Houghton, Peter J: 28 articles (02/2022 - 08/2007)

Drugs and Biologics

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

1.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 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." 01/01/1988 - "Recent improvements in survival of patients with Ewing's sarcoma have been made since the addition of cyclophosphamide-based adjuvant chemotherapy to primary surgery and radiation. " 10/01/1962 - "Cyclophosphamide therapy in children with Ewing's sarcoma." 03/01/2011 - "sFLT01 in combination with cyclophosphamide resulted in greater inhibition of tumor growth than either agent used alone as a monotherapy in the A673 Ewing's sarcoma model. "
2.	Vincristine (Oncovin)FDA LinkGeneric 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. " 01/01/2023 - "This patient's history of therapy for Ewing sarcoma prompted consideration of medication-induced vasospastic changes most likely related to the adverse vasospastic effects of vincristine. " 09/01/2018 - "Oesophageal narrowing during combination chemotherapy in Ewing's sarcoma: Is vincristine a culprit?" 11/01/2001 - "Six hundred ninety Ewing tumor patients were treated between 1992 and 1999 with local therapy and vincristine. " 06/03/2020 - "Up-regulation of GLI1 in vincristine-resistant rhabdomyosarcoma and Ewing sarcoma."
3.	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." 11/01/2022 - "This single-center, retrospective review describes the tolerability of continuous infusion and bolus administration of ifosfamide in 10 pediatric patients with Ewing sarcoma. " 04/01/2015 - "Ifosfamide dose-intensification for patients with metastatic Ewing sarcoma." 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. "
4.	Doxorubicin (Adriamycin)FDA LinkGeneric 11/09/2022 - "Identification of Factors Driving Doxorubicin-Resistant Ewing Tumor Cells to Survival." 08/01/2020 - "The other two patients were males who had Ewing's sarcoma who developed CCMP following treatment with doxorubicin." 07/26/2016 - "Effective molecular targeting of CDK4/6 and IGF-1R in a rare FUS-ERG fusion CDKN2A-deletion doxorubicin-resistant Ewing's sarcoma patient-derived orthotopic xenograft (PDOX) nude-mouse model." 01/01/2015 - "CD99 triggering in Ewing sarcoma delivers a lethal signal through p53 pathway reactivation and cooperates with doxorubicin." 01/01/2006 - "Targeting CD99 in association with doxorubicin: an effective combined treatment for Ewing's sarcoma."
5.	Etoposide (VP 16)FDA LinkGeneric 03/17/2022 - "Pan-inhibitor screening indicated that etoposide, a drug used for Ewing sarcoma treatment, promotes transcription of pncCCND1_B and repression of CCND1 expression. " 01/01/2018 - "Ewing sarcoma is highly sensitive to genotoxic agents such as etoposide, but the underlying molecular basis of this sensitivity is unclear. " 01/01/2016 - "The records of all patients treated at our department between 1989 and 2012 for relapsing/refractory Ewing sarcoma who received oral VP-16 were analyzed. " 01/01/2016 - "To assess the efficacy and toxicity of low-dose oral etoposide (VP) 16 in relapsing/refractory Ewing sarcoma. " 01/01/2016 - "Oral etoposide in relapsed or refractory Ewing sarcoma: a monoinstitutional experience in children and adolescents."
6.	Irinotecan (Camptosar)FDA LinkGeneric 03/01/2014 - "Based on a preclinical rationale of responses in xenografts and backed by a phase I study in children, the Euro-E.W.I.N.G consortium planned a phase II window study of irinotecan in newly diagnosed high risk metastatic patients with Ewing sarcoma. " 03/01/2014 - "A phase II window study of irinotecan (CPT-11) in high risk Ewing sarcoma: a Euro-E.W.I.N.G. " 01/01/2023 - "Comment on: Irinotecan dose schedule for the treatment of Ewing sarcoma-protracted/metronomic schedule-wording matters!" 01/01/2023 - "In the treatment of recurrent Ewing sarcoma, investigators should consider the use of a 10-day schedule for administration of irinotecan." 01/01/2023 - "Irinotecan dose schedule for the treatment of Ewing sarcoma."
7.	EWS-FLI fusion proteinIBA 04/01/2019 - "Preclinical and clinical studies showed promising results, with the most robust evidences of PARPi efficacy obtained on Ewing sarcoma bearing EWS-FLI1 or EWS-ERG genomic fusions. " 01/01/2019 - "These studies uncover a fine-tuned modulation of the proto-oncogene CCND1 in Ewing sarcoma cells via alternative complexes formed by DHX9 with either EWS-FLI1 or pncCCND1_B-Sam68. " 12/23/2016 - "Our study provides novel insights into EWS-FLI1 turnover, a critical pathway in Ewing sarcoma pathogenesis, and lays new ground to develop novel therapeutic strategies in Ewing sarcoma." 03/01/2016 - "Up to now very few global quantitative proteomic studies have been published to help understand the oncogenic transformation induced by EWS-FLI1 fusion protein and leading to Ewing sarcoma development and dissemination. " 12/26/2014 - "Synthesis and structure-activity relationship studies of small molecule disruptors of EWS-FLI1 interactions in Ewing's sarcoma."
8.	AnthracyclinesIBA 09/04/2014 - "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. " 09/01/2012 - "The authors report a case of a 19-year-old man developing HF after anthracycline treatment for Ewing sarcoma. " 01/19/2011 - "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. " 01/21/2009 - "For both Wilms' tumour and Ewing's sarcoma a significant difference in survival in favour of treatment with anthracyclines was identified. " 06/05/2023 - "While anthracycline therapy has been shown to improve outcomes in Ewing sarcoma, it may be associated with severe and even fatal cardiac dysfunction. "
9.	Dactinomycin (Actinomycin)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. " 08/01/2022 - "Efficacy of replacing actinomycin-D with carboplatin in Ewing sarcoma consolidation treatment: Single-center experience." 03/01/1989 - "The first Intergroup Ewing's Sarcoma Study, completed in 1978, demonstrated that adjuvant therapy with vincristine, dactinomycin was superior to VAC alone or VAC with pulmonary irradiation. " 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. "
10.	Small Interfering RNA (siRNA)IBA 03/01/2015 - "With siRNA/ND-H complexes, we measured a high inhibition efficacy of EWS/FLI-1 gene expression in Ewing sarcoma cell line. " 01/01/2016 - "To study the role of DDX3 in Ewing sarcoma, we generated stable DDX3-knockdown Ewing sarcoma cell lines using DDX3-specific small hairpin RNA (shRNA), and assessed oncogenic activity. " 07/01/2004 - "In this study we report that synthetic small interfering RNA (siRNA) specifically suppresses EWS/FLI-1 fusion gene expression in SK-ES Ewing's sarcoma cells. " 11/12/2021 - "The functional characterization of FEZF1 was performed in three Ewing sarcoma cell lines (A673, SK-N-MC, SK-ES-1) through an shRNA-directed silencing approach. " 05/27/2021 - "As a proof-of-principle experiment, we quantified the biodistribution and aggregation state of new cationic FNDs capable of transporting small interfering RNA inhibiting the oncogene responsible for Ewing sarcoma. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 06/01/2016 - "Most studies from western countries have reported improvement in outcomes following multi-agent chemotherapy, with no difference in outcome between skeletal and extraskeletal Ewing sarcoma. " 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. " 08/01/2016 - "Currently, the optimal management of pelvic Ewing's sarcoma is contro-versial but our study shows a trend for improved -survival for patients treated with chemotherapy and surgery."
2.	Therapeutics 12/01/2003 - "We propose that low-dose maintenance therapy may be beneficial in the subgroup of Ewing's tumor patients with late relapse after HDC." 05/15/2019 - "Although multidisciplinary therapies have improved local control and overall survival in Ewing sarcoma (ES), the prognosis of pelvic lesions remains markedly worse than that of limb ES. " 09/01/1997 - "Although these results represent improvement in outcome for our patients, more effective therapy is needed for children with Ewing sarcoma, especially those with metastatic disease at presentation." 08/06/2013 - "Novel treatment strategies in Ewing sarcoma include targeted cellular therapies. " 10/01/1999 - "The prognosis of Ewing's tumor patients has been improved gradually through cooperative therapy studies, so that meanwhile 55 to 65% of the patients survive relapse-free in the long term. "
3.	Radiotherapy 01/01/2011 - "Role of radiation therapy in the multidisciplinary management of Ewing's Sarcoma of bone in pediatric patients: An effective treatment for local control." 12/01/2019 - "Radiation therapy only improved survival for Ewing sarcoma. " 02/01/2018 - "The aim of this study was to analyse a group of patients with non-metastatic Ewing's sarcoma at presentation and identify prognostic factors affecting the development of local recurrence, in order to assess the role of 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. "
4.	Adjuvant Chemotherapy 09/01/2018 - "In this article, we describe a 12-year-old Hispanic female cured of localized, cutaneous Ewing sarcoma (pT1aN0M0) at the 40-month follow-up following surgical resection and adjuvant chemotherapy according to the COG AEWS1031 protocol for Ewing sarcoma of the bone. " 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." 04/01/2023 - "The pathology was suggestive of Ewings sarcoma and she received adjuvant chemotherapy. " 01/01/2023 - "Non-chemotherapy adjuvant agents in TP53 mutant Ewing sarcoma."
5.	Neoadjuvant Therapy 01/01/2020 - "This network meta-analysis aimed at comparing the efficacy of local control strategies after neoadjuvant chemotherapy in patients with Ewing sarcoma. " 01/01/2020 - "Efficacy of Local Control Strategies for Ewing Sarcoma After Neoadjuvant Chemotherapy: A Network Meta-analysis." 09/21/2022 - "This study was conducted to evaluate the clinical applicability of integrated PET/MRI for staging and monitoring the effectiveness of neoadjuvant chemotherapy in Ewing sarcoma patients. " 01/01/2022 - "This study focuses on MRI radiomics of skeletal Ewing sarcoma and aims to investigate feature reproducibility and machine learning prediction of response to neoadjuvant chemotherapy. " 01/01/2022 - "Compared to 2D approach, 3D MRI radiomics of Ewing sarcoma had superior reproducibility and higher accuracy in predicting response to neoadjuvant chemotherapy, particularly when using logistic regression classifier."