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Unresectable Ewing Sarcoma

MedGen UID:
1387874
•Concept ID:
C4331474
•
Neoplastic Process

Definition

A Ewing sarcoma which is not amenable to surgical resection. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Radiotherapy in bone sarcoma: the quest for better treatment option.
Locquet MA, Brahmi M, Blay JY, Dutour A
BMC Cancer 2023 Aug 11;23(1):742. doi: 10.1186/s12885-023-11232-3. PMID: 37563551Free PMC Article
Receptor Tyrosine Kinase Inhibitors for the Treatment of Recurrent and Unresectable Bone Sarcomas.
Albarrán V, Villamayor ML, Chamorro J, Rosero DI, Pozas J, San Román M, Calvo JC, Pérez de Aguado P, Moreno J, Guerrero P, González C, García de Quevedo C, Álvarez-Ballesteros P, Vaz MÁ
Int J Mol Sci 2022 Nov 9;23(22) doi: 10.3390/ijms232213784. PMID: 36430263Free PMC Article
Head and neck sarcomas in adulthood: current trends and evolving management concepts.
Kalavrezos N, Sinha D
Br J Oral Maxillofac Surg 2020 Oct;58(8):890-897. Epub 2020 Jul 4 doi: 10.1016/j.bjoms.2020.05.015. PMID: 32636095

Recent clinical studies

Etiology

Protocol for the 2ND-STEP study, Japan Clinical Oncology Group study JCOG1802: a randomized phase II trial of second-line treatment for advanced soft tissue sarcoma comparing trabectedin, eribulin and pazopanib.
Endo M, Kataoka T, Fujiwara T, Tsukushi S, Takahashi M, Kobayashi E, Yamada Y, Tanaka T, Nezu Y, Hiraga H, Wasa J, Nagano A, Nakano K, Nakayama R, Hamada T, Kawano M, Torigoe T, Sakamoto A, Asanuma K, Morii T, Machida R, Sekino Y, Fukuda H, Oda Y, Ozaki T, Tanaka K
BMC Cancer 2023 Mar 8;23(1):219. doi: 10.1186/s12885-023-10693-w. PMID: 36890471Free PMC Article
Receptor Tyrosine Kinase Inhibitors for the Treatment of Recurrent and Unresectable Bone Sarcomas.
Albarrán V, Villamayor ML, Chamorro J, Rosero DI, Pozas J, San Román M, Calvo JC, Pérez de Aguado P, Moreno J, Guerrero P, González C, García de Quevedo C, Álvarez-Ballesteros P, Vaz MÁ
Int J Mol Sci 2022 Nov 9;23(22) doi: 10.3390/ijms232213784. PMID: 36430263Free PMC Article
Systemic Therapy for Chondrosarcoma.
Rock A, Ali S, Chow WA
Curr Treat Options Oncol 2022 Feb;23(2):199-209. Epub 2022 Feb 21 doi: 10.1007/s11864-022-00951-7. PMID: 35190971
Systemic treatments and outcomes in CIC-rearranged Sarcoma: A national multi-centre clinicopathological series and literature review.
Connolly EA, Bhadri VA, Wake J, Ingley KM, Lewin J, Bae S, Wong DD, Long AP, Pryor D, Thompson SR, Strach MC, Grimison PS, Mahar A, Bonar F, Maclean F, Hong A
Cancer Med 2022 Apr;11(8):1805-1816. Epub 2022 Feb 17 doi: 10.1002/cam4.4580. PMID: 35178869Free PMC Article
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article

Diagnosis

Protocol for the 2ND-STEP study, Japan Clinical Oncology Group study JCOG1802: a randomized phase II trial of second-line treatment for advanced soft tissue sarcoma comparing trabectedin, eribulin and pazopanib.
Endo M, Kataoka T, Fujiwara T, Tsukushi S, Takahashi M, Kobayashi E, Yamada Y, Tanaka T, Nezu Y, Hiraga H, Wasa J, Nagano A, Nakano K, Nakayama R, Hamada T, Kawano M, Torigoe T, Sakamoto A, Asanuma K, Morii T, Machida R, Sekino Y, Fukuda H, Oda Y, Ozaki T, Tanaka K
BMC Cancer 2023 Mar 8;23(1):219. doi: 10.1186/s12885-023-10693-w. PMID: 36890471Free PMC Article
Long-term follow-up results of pediatric and adolescent patients with localized Ewing sarcoma treated based on stratified modalities.
Zeng C, Chen T, Wang J, Sun F, Huang J, Lu S, Zhu J, Zhang Y, Sun X, Zhen Z
Cancer Med 2023 Apr;12(8):9409-9419. Epub 2023 Feb 21 doi: 10.1002/cam4.5703. PMID: 36808251Free PMC Article
Systemic Therapy for Chondrosarcoma.
Rock A, Ali S, Chow WA
Curr Treat Options Oncol 2022 Feb;23(2):199-209. Epub 2022 Feb 21 doi: 10.1007/s11864-022-00951-7. PMID: 35190971
Systemic treatments and outcomes in CIC-rearranged Sarcoma: A national multi-centre clinicopathological series and literature review.
Connolly EA, Bhadri VA, Wake J, Ingley KM, Lewin J, Bae S, Wong DD, Long AP, Pryor D, Thompson SR, Strach MC, Grimison PS, Mahar A, Bonar F, Maclean F, Hong A
Cancer Med 2022 Apr;11(8):1805-1816. Epub 2022 Feb 17 doi: 10.1002/cam4.4580. PMID: 35178869Free PMC Article
Head and neck sarcomas in adulthood: current trends and evolving management concepts.
Kalavrezos N, Sinha D
Br J Oral Maxillofac Surg 2020 Oct;58(8):890-897. Epub 2020 Jul 4 doi: 10.1016/j.bjoms.2020.05.015. PMID: 32636095

Therapy

Protocol for the 2ND-STEP study, Japan Clinical Oncology Group study JCOG1802: a randomized phase II trial of second-line treatment for advanced soft tissue sarcoma comparing trabectedin, eribulin and pazopanib.
Endo M, Kataoka T, Fujiwara T, Tsukushi S, Takahashi M, Kobayashi E, Yamada Y, Tanaka T, Nezu Y, Hiraga H, Wasa J, Nagano A, Nakano K, Nakayama R, Hamada T, Kawano M, Torigoe T, Sakamoto A, Asanuma K, Morii T, Machida R, Sekino Y, Fukuda H, Oda Y, Ozaki T, Tanaka K
BMC Cancer 2023 Mar 8;23(1):219. doi: 10.1186/s12885-023-10693-w. PMID: 36890471Free PMC Article
Receptor Tyrosine Kinase Inhibitors for the Treatment of Recurrent and Unresectable Bone Sarcomas.
Albarrán V, Villamayor ML, Chamorro J, Rosero DI, Pozas J, San Román M, Calvo JC, Pérez de Aguado P, Moreno J, Guerrero P, González C, García de Quevedo C, Álvarez-Ballesteros P, Vaz MÁ
Int J Mol Sci 2022 Nov 9;23(22) doi: 10.3390/ijms232213784. PMID: 36430263Free PMC Article
Radiation Therapy Dose Escalation in Unresectable Ewing Sarcoma: Final Results of a Phase 3 Randomized Controlled Trial.
Laskar S, Sinha S, Chatterjee A, Khanna N, Manjali JJ, Puri A, Gulia A, Nayak P, Vora T, Chinnaswamy G, Prasad M, Bajpai J, Juvekar S, Desai S, Janu A, Rangarajan V, Purandare N, Shah S, Rekhi B, Jambhekar N, Muckaden MA, Kurkure P
Int J Radiat Oncol Biol Phys 2022 Aug 1;113(5):996-1002. Epub 2022 May 11 doi: 10.1016/j.ijrobp.2022.04.024. PMID: 35568246
Systemic Therapy for Chondrosarcoma.
Rock A, Ali S, Chow WA
Curr Treat Options Oncol 2022 Feb;23(2):199-209. Epub 2022 Feb 21 doi: 10.1007/s11864-022-00951-7. PMID: 35190971
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article

Prognosis

Long-term follow-up results of pediatric and adolescent patients with localized Ewing sarcoma treated based on stratified modalities.
Zeng C, Chen T, Wang J, Sun F, Huang J, Lu S, Zhu J, Zhang Y, Sun X, Zhen Z
Cancer Med 2023 Apr;12(8):9409-9419. Epub 2023 Feb 21 doi: 10.1002/cam4.5703. PMID: 36808251Free PMC Article
Systemic treatments and outcomes in CIC-rearranged Sarcoma: A national multi-centre clinicopathological series and literature review.
Connolly EA, Bhadri VA, Wake J, Ingley KM, Lewin J, Bae S, Wong DD, Long AP, Pryor D, Thompson SR, Strach MC, Grimison PS, Mahar A, Bonar F, Maclean F, Hong A
Cancer Med 2022 Apr;11(8):1805-1816. Epub 2022 Feb 17 doi: 10.1002/cam4.4580. PMID: 35178869Free PMC Article
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article
Local therapy in non-metastatic primary Ewing sarcoma of the mandible and maxilla in children.
Qureshi SS, Bhagat M, Laskar S, Kembhavi S, Vora T, Ramadwar M, Chinnaswamy G, Prasad M, Khanna N, Shah S, Talole S
Int J Oral Maxillofac Surg 2016 Aug;45(8):938-44. Epub 2016 Mar 26 doi: 10.1016/j.ijom.2016.03.004. PMID: 27026058
Ewing's sarcoma of the head and neck in children.
Vaccani JP, Forte V, de Jong AL, Taylor G
Int J Pediatr Otorhinolaryngol 1999 May 25;48(3):209-16. doi: 10.1016/s0165-5876(99)00030-0. PMID: 10402117

Clinical prediction guides

Radiation Therapy Dose Escalation in Unresectable Ewing Sarcoma: Final Results of a Phase 3 Randomized Controlled Trial.
Laskar S, Sinha S, Chatterjee A, Khanna N, Manjali JJ, Puri A, Gulia A, Nayak P, Vora T, Chinnaswamy G, Prasad M, Bajpai J, Juvekar S, Desai S, Janu A, Rangarajan V, Purandare N, Shah S, Rekhi B, Jambhekar N, Muckaden MA, Kurkure P
Int J Radiat Oncol Biol Phys 2022 Aug 1;113(5):996-1002. Epub 2022 May 11 doi: 10.1016/j.ijrobp.2022.04.024. PMID: 35568246
Systemic Therapy for Chondrosarcoma.
Rock A, Ali S, Chow WA
Curr Treat Options Oncol 2022 Feb;23(2):199-209. Epub 2022 Feb 21 doi: 10.1007/s11864-022-00951-7. PMID: 35190971
EphA2 Expression in Bone Sarcomas: Bioinformatic Analyses and Preclinical Characterization in Patient-Derived Models of Osteosarcoma, Ewing's Sarcoma and Chondrosarcoma.
Giordano G, Merlini A, Ferrero G, Mesiano G, Fiorino E, Brusco S, Centomo ML, Leuci V, D'Ambrosio L, Aglietta M, Sangiolo D, Grignani G, Pignochino Y
Cells 2021 Oct 26;10(11) doi: 10.3390/cells10112893. PMID: 34831119Free PMC Article
Efficacy of carbon-ion radiotherapy and high-dose chemotherapy for patients with unresectable Ewing's sarcoma family of tumors.
Iwata S, Yonemoto T, Ishii T, Kumagai K, Imai R, Hagiwara Y, Kamada T, Tatezaki S
Int J Clin Oncol 2013 Dec;18(6):1114-8. Epub 2012 Oct 3 doi: 10.1007/s10147-012-0480-y. PMID: 23053401
Phase II study of sequential gemcitabine followed by docetaxel for recurrent Ewing sarcoma, osteosarcoma, or unresectable or locally recurrent chondrosarcoma: results of Sarcoma Alliance for Research Through Collaboration Study 003.
Fox E, Patel S, Wathen JK, Schuetze S, Chawla S, Harmon D, Reinke D, Chugh R, Benjamin RS, Helman LJ
Oncologist 2012;17(3):321. Epub 2012 Feb 23 doi: 10.1634/theoncologist.2010-0265. PMID: 22363068Free PMC Article

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