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Refractory Ewing Sarcoma

MedGen UID:
1631504
•Concept ID:
C4684866
•
Neoplastic Process

Definition

Ewing sarcoma that does not respond to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory Ewing Sarcoma

Professional guidelines

PubMed

Current Role of Topoisomerase I Inhibitors for the Treatment of Mesenchymal Malignancies and Their Potential Future Use as Payload of Sarcoma-Specific Antibody-Drug Conjugates.
Schöffski P, Wang CC, Schöffski MP, Wozniak A
Oncol Res Treat 2024;47(1-2):18-41. Epub 2023 Nov 28 doi: 10.1159/000535491. PMID: 38016427Free PMC Article

Recent clinical studies

Etiology

Longer versus Shorter Schedules of Vincristine, Irinotecan, and Temozolomide (VIT) for Relapsed or Refractory Ewing Sarcoma: A Randomized Controlled Phase 2 Trial.
Xu J, Xie L, Sun X, Liu K, Liang X, Cai Z, Tang X, Guo W
Clin Cancer Res 2023 Mar 14;29(6):1040-1046. doi: 10.1158/1078-0432.CCR-22-3546. PMID: 36622702
T-cell replete haploidentical stem cell transplantation with low dose anti-thymocyte globulin for relapsed/refractory Ewing sarcoma family tumors.
Sano H, Mochizuki K, Kobayashi S, Ohara Y, Takahashi N, Kudo S, Ikeda K, Ohto H, Kikuta A
Cancer Rep (Hoboken) 2022 Jul;5(7):e1519. Epub 2021 Jul 22 doi: 10.1002/cnr2.1519. PMID: 34291886Free PMC Article
Oral etoposide in relapsed or refractory Ewing sarcoma: a monoinstitutional experience in children and adolescents.
Podda MG, Luksch R, Puma N, Gandola L, Morosi C, Terenziani M, Ferrari A, Casanova M, Spreafico F, Meazza C, Catania S, Schiavello E, Biassoni V, Chiaravalli S, Massimino M
Tumori 2016 Jan-Feb;102(1):84-8. Epub 2015 Oct 28 doi: 10.5301/tj.5000419. PMID: 26797935
Etoposide and carbo-or cisplatin combination therapy in refractory or relapsed Ewing sarcoma: a large retrospective study.
van Maldegem AM, Benson C, Rutkowski P, Blay JY, van den Berg H, Placzke J, Rasper M, Judson I, Juergens H, Dirksen U, Gelderblom H
Pediatr Blood Cancer 2015 Jan;62(1):40-4. Epub 2014 Sep 22 doi: 10.1002/pbc.25230. PMID: 25251256
Vincristine, irinotecan, and temozolomide in patients with relapsed and refractory Ewing sarcoma.
Raciborska A, Bilska K, Drabko K, Chaber R, Pogorzala M, Wyrobek E, Polczyńska K, Rogowska E, Rodriguez-Galindo C, Wozniak W
Pediatr Blood Cancer 2013 Oct;60(10):1621-5. Epub 2013 Jun 15 doi: 10.1002/pbc.24621. PMID: 23776128

Diagnosis

Surface and Global Proteome Analyses Identify ENPP1 and Other Surface Proteins as Actionable Immunotherapeutic Targets in Ewing Sarcoma.
Mooney B, Negri GL, Shyp T, Delaidelli A, Zhang HF, Spencer Miko SE, Weiner AK, Radaoui AB, Shraim R, Lizardo MM, Hughes CS, Li A, El-Naggar AM, Rouleau M, Li W, Dimitrov DS, Kurmasheva RT, Houghton PJ, Diskin SJ, Maris JM, Morin GB, Sorensen PH
Clin Cancer Res 2024 Mar 1;30(5):1022-1037. doi: 10.1158/1078-0432.CCR-23-2187. PMID: 37812652Free PMC Article
Preclinical Testing of an Oncolytic Parvovirus in Ewing Sarcoma: Protoparvovirus H-1 Induces Apoptosis and Lytic Infection In Vitro but Fails to Improve Survival In Vivo.
Lacroix J, Kis Z, Josupeit R, Schlund F, Stroh-Dege A, Frank-Stöhr M, Leuchs B, Schlehofer JR, Rommelaere J, Dinsart C
Viruses 2018 Jun 3;10(6) doi: 10.3390/v10060302. PMID: 29865280Free PMC Article
Oral etoposide in relapsed or refractory Ewing sarcoma: a monoinstitutional experience in children and adolescents.
Podda MG, Luksch R, Puma N, Gandola L, Morosi C, Terenziani M, Ferrari A, Casanova M, Spreafico F, Meazza C, Catania S, Schiavello E, Biassoni V, Chiaravalli S, Massimino M
Tumori 2016 Jan-Feb;102(1):84-8. Epub 2015 Oct 28 doi: 10.5301/tj.5000419. PMID: 26797935
Etoposide and carbo-or cisplatin combination therapy in refractory or relapsed Ewing sarcoma: a large retrospective study.
van Maldegem AM, Benson C, Rutkowski P, Blay JY, van den Berg H, Placzke J, Rasper M, Judson I, Juergens H, Dirksen U, Gelderblom H
Pediatr Blood Cancer 2015 Jan;62(1):40-4. Epub 2014 Sep 22 doi: 10.1002/pbc.25230. PMID: 25251256
Diagnostic challenge in recurrent skin rash after autologous bone marrow transplantation.
Inaba H, Hale G, Leung W, Woodard P, Burnette K, Handgretinger R, Barfield R
J Pediatr Hematol Oncol 2006 Aug;28(8):525-8. doi: 10.1097/01.mph.0000212966.60383.74. PMID: 16912592

Therapy

Longer versus Shorter Schedules of Vincristine, Irinotecan, and Temozolomide (VIT) for Relapsed or Refractory Ewing Sarcoma: A Randomized Controlled Phase 2 Trial.
Xu J, Xie L, Sun X, Liu K, Liang X, Cai Z, Tang X, Guo W
Clin Cancer Res 2023 Mar 14;29(6):1040-1046. doi: 10.1158/1078-0432.CCR-22-3546. PMID: 36622702
High-dose Chemotherapy Response in Adults with Relapsed/Refractory Small Round Cell Tumours.
Aykan MB, Erturk I, Acar R, Yildiran GS, Yildiz B, Karadurmus N
J Coll Physicians Surg Pak 2022 Jan;32(1):51-56. doi: 10.29271/jcpsp.2022.01.51. PMID: 34983148
T-cell replete haploidentical stem cell transplantation with low dose anti-thymocyte globulin for relapsed/refractory Ewing sarcoma family tumors.
Sano H, Mochizuki K, Kobayashi S, Ohara Y, Takahashi N, Kudo S, Ikeda K, Ohto H, Kikuta A
Cancer Rep (Hoboken) 2022 Jul;5(7):e1519. Epub 2021 Jul 22 doi: 10.1002/cnr2.1519. PMID: 34291886Free PMC Article
Etoposide and carbo-or cisplatin combination therapy in refractory or relapsed Ewing sarcoma: a large retrospective study.
van Maldegem AM, Benson C, Rutkowski P, Blay JY, van den Berg H, Placzke J, Rasper M, Judson I, Juergens H, Dirksen U, Gelderblom H
Pediatr Blood Cancer 2015 Jan;62(1):40-4. Epub 2014 Sep 22 doi: 10.1002/pbc.25230. PMID: 25251256
Vincristine, irinotecan, and temozolomide in patients with relapsed and refractory Ewing sarcoma.
Raciborska A, Bilska K, Drabko K, Chaber R, Pogorzala M, Wyrobek E, Polczyńska K, Rogowska E, Rodriguez-Galindo C, Wozniak W
Pediatr Blood Cancer 2013 Oct;60(10):1621-5. Epub 2013 Jun 15 doi: 10.1002/pbc.24621. PMID: 23776128

Prognosis

T-cell replete haploidentical stem cell transplantation with low dose anti-thymocyte globulin for relapsed/refractory Ewing sarcoma family tumors.
Sano H, Mochizuki K, Kobayashi S, Ohara Y, Takahashi N, Kudo S, Ikeda K, Ohto H, Kikuta A
Cancer Rep (Hoboken) 2022 Jul;5(7):e1519. Epub 2021 Jul 22 doi: 10.1002/cnr2.1519. PMID: 34291886Free PMC Article
Etoposide and carbo-or cisplatin combination therapy in refractory or relapsed Ewing sarcoma: a large retrospective study.
van Maldegem AM, Benson C, Rutkowski P, Blay JY, van den Berg H, Placzke J, Rasper M, Judson I, Juergens H, Dirksen U, Gelderblom H
Pediatr Blood Cancer 2015 Jan;62(1):40-4. Epub 2014 Sep 22 doi: 10.1002/pbc.25230. PMID: 25251256
A study of docetaxel and irinotecan in children and young adults with recurrent or refractory Ewing sarcoma family of tumors.
Yoon JH, Kwon MM, Park HJ, Park SY, Lim KY, Joo J, Park BK
BMC Cancer 2014 Aug 28;14:622. doi: 10.1186/1471-2407-14-622. PMID: 25164234Free PMC Article
Survival and prognostic factors in adult patients with recurrent or refractory ewing sarcoma family tumours: a 13-years retrospective study in Turkey.
Yildiz I, Sen F, Ekenel M, Darendeliler E, Bavbek S, Agaoglu F, Ozger H, Eralp L, Bilgic B, Basaran M
In Vivo 2014 May-Jun;28(3):403-9. PMID: 24815845
Vincristine, irinotecan, and temozolomide in patients with relapsed and refractory Ewing sarcoma.
Raciborska A, Bilska K, Drabko K, Chaber R, Pogorzala M, Wyrobek E, Polczyńska K, Rogowska E, Rodriguez-Galindo C, Wozniak W
Pediatr Blood Cancer 2013 Oct;60(10):1621-5. Epub 2013 Jun 15 doi: 10.1002/pbc.24621. PMID: 23776128

Clinical prediction guides

Current Role of Topoisomerase I Inhibitors for the Treatment of Mesenchymal Malignancies and Their Potential Future Use as Payload of Sarcoma-Specific Antibody-Drug Conjugates.
Schöffski P, Wang CC, Schöffski MP, Wozniak A
Oncol Res Treat 2024;47(1-2):18-41. Epub 2023 Nov 28 doi: 10.1159/000535491. PMID: 38016427Free PMC Article
High-dose Chemotherapy Response in Adults with Relapsed/Refractory Small Round Cell Tumours.
Aykan MB, Erturk I, Acar R, Yildiran GS, Yildiz B, Karadurmus N
J Coll Physicians Surg Pak 2022 Jan;32(1):51-56. doi: 10.29271/jcpsp.2022.01.51. PMID: 34983148
Preclinical Testing of an Oncolytic Parvovirus in Ewing Sarcoma: Protoparvovirus H-1 Induces Apoptosis and Lytic Infection In Vitro but Fails to Improve Survival In Vivo.
Lacroix J, Kis Z, Josupeit R, Schlund F, Stroh-Dege A, Frank-Stöhr M, Leuchs B, Schlehofer JR, Rommelaere J, Dinsart C
Viruses 2018 Jun 3;10(6) doi: 10.3390/v10060302. PMID: 29865280Free PMC Article
A phase I/II trial and pharmacokinetic study of mithramycin in children and adults with refractory Ewing sarcoma and EWS-FLI1 fusion transcript.
Grohar PJ, Glod J, Peer CJ, Sissung TM, Arnaldez FI, Long L, Figg WD, Whitcomb P, Helman LJ, Widemann BC
Cancer Chemother Pharmacol 2017 Sep;80(3):645-652. Epub 2017 Jul 22 doi: 10.1007/s00280-017-3382-x. PMID: 28735378Free PMC Article
Phase II study of olaparib in patients with refractory Ewing sarcoma following failure of standard chemotherapy.
Choy E, Butrynski JE, Harmon DC, Morgan JA, George S, Wagner AJ, D'Adamo D, Cote GM, Flamand Y, Benes CH, Haber DA, Baselga JM, Demetri GD
BMC Cancer 2014 Nov 5;14:813. doi: 10.1186/1471-2407-14-813. PMID: 25374341Free PMC Article

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