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Localized Extraskeletal Ewing Sarcoma

MedGen UID:
163505
•Concept ID:
C0855043
•
Neoplastic Process
Synonyms: Extra-Osseous Ewing's Sarcoma, Nonmetastatic; Non-Metastatic Extra-Osseous Ewing's Sarcoma; Non-Metastatic Extraosseous Ewing Sarcoma; Non-Metastatic Extraosseous Ewing's Sarcoma; Non-Metastatic Extraskeletal Ewing Sarcoma; Non-Metastatic Extraskeletal Ewing's Sarcoma

Definition

Ewing sarcoma that arises from the soft tissue and is confined to a specific site without evidence of spread to other anatomic sites. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLocalized Extraskeletal Ewing Sarcoma

Professional guidelines

PubMed

Evaluating the Soft Tissue Sarcoma Paradigm for the Local Management of Extraskeletal Ewing Sarcoma.
Boyce-Fappiano D, Guadagnolo BA, Ratan R, Wang WL, Wagner MJ, Patel S, Livingston JA, Lin PP, Diao K, Mitra D, Farooqi A, Lazar AJ, Roland CL, Bishop AJ
Oncologist 2021 Mar;26(3):250-260. Epub 2020 Dec 14 doi: 10.1002/onco.13616. PMID: 33289298Free PMC Article

Recent clinical studies

Etiology

Treatment outcomes of extraskeletal Ewing sarcoma.
Houdek MT, Heidenreich MJ, Ahmed SK, Allen-Rhoades W, Siontis BL, Robinson SI, Petersen IA, Rose PS
J Surg Oncol 2023 Jul;128(1):105-110. Epub 2023 Mar 31 doi: 10.1002/jso.27250. PMID: 36999583
Prognostic Factors and Clinical Outcomes in Extraskeletal Ewing Sarcoma: A Cohort Study.
Mathew J, Arjunan R, Dasappa A, Namachivayam A
Ann Surg Oncol 2023 May;30(5):3084-3094. Epub 2022 Dec 23 doi: 10.1245/s10434-022-12992-1. PMID: 36564656
Extraskeletal Ewing Sarcoma from Head to Toe: Multimodality Imaging Review.
Wright A, Desai M, Bolan CW, Badawy M, Guccione J, Rao Korivi B, Pickhardt PJ, Mellnick VM, Lubner MG, Chen L, Elsayes KM
Radiographics 2022 Jul-Aug;42(4):1145-1160. Epub 2022 May 27 doi: 10.1148/rg.210226. PMID: 35622491
Ewing's sarcoma of the thumb.
Yamaguchi T, Tamai K, Saotome K, Hoshino T, Masawa N
Skeletal Radiol 1997 Dec;26(12):725-8. doi: 10.1007/s002560050319. PMID: 9453107
Malignant neuroepithelioma (peripheral neuroblastoma). A clinicopathologic study of 15 cases.
Hashimoto H, Enjoji M, Nakajima T, Kiryu H, Daimaru Y
Am J Surg Pathol 1983 Jun;7(4):309-18. doi: 10.1097/00000478-198306000-00002. PMID: 6869663

Diagnosis

Gastrointestinal Ewing Sarcoma: A Clinicopathological and Molecular Genetic Analysis of 25 Cases.
Yin T, Shao M, Sun M, Zhao L, Lao IW, Yu L, Wang J
Am J Surg Pathol 2024 Mar 1;48(3):275-283. Epub 2023 Dec 8 doi: 10.1097/PAS.0000000000002163. PMID: 38062799
Prognostic Factors and Clinical Outcomes in Extraskeletal Ewing Sarcoma: A Cohort Study.
Mathew J, Arjunan R, Dasappa A, Namachivayam A
Ann Surg Oncol 2023 May;30(5):3084-3094. Epub 2022 Dec 23 doi: 10.1245/s10434-022-12992-1. PMID: 36564656
Extraskeletal Ewing Sarcoma from Head to Toe: Multimodality Imaging Review.
Wright A, Desai M, Bolan CW, Badawy M, Guccione J, Rao Korivi B, Pickhardt PJ, Mellnick VM, Lubner MG, Chen L, Elsayes KM
Radiographics 2022 Jul-Aug;42(4):1145-1160. Epub 2022 May 27 doi: 10.1148/rg.210226. PMID: 35622491
Extraskeletal Ewing sarcoma of the buccal space.
Spaulding SL, Xing MH, Seo GT, Matloob A, Khorsandi AS, Urken ML
Clin Imaging 2021 May;73:108-110. Epub 2020 Dec 25 doi: 10.1016/j.clinimag.2020.12.004. PMID: 33360836
Extraskeletal Ewing's sarcoma: a report of 18 cases and literature review.
Xie CF, Liu MZ, Xi M
Chin J Cancer 2010 Apr;29(4):420-4. doi: 10.5732/cjc.009.10402. PMID: 20346219

Therapy

Treatment outcomes of extraskeletal Ewing sarcoma.
Houdek MT, Heidenreich MJ, Ahmed SK, Allen-Rhoades W, Siontis BL, Robinson SI, Petersen IA, Rose PS
J Surg Oncol 2023 Jul;128(1):105-110. Epub 2023 Mar 31 doi: 10.1002/jso.27250. PMID: 36999583
Prognostic Factors and Clinical Outcomes in Extraskeletal Ewing Sarcoma: A Cohort Study.
Mathew J, Arjunan R, Dasappa A, Namachivayam A
Ann Surg Oncol 2023 May;30(5):3084-3094. Epub 2022 Dec 23 doi: 10.1245/s10434-022-12992-1. PMID: 36564656
Extraosseous Ewing sarcoma in children and adolescents: A retrospective series from a referral pediatric oncology center.
Livellara V, Bergamaschi L, Puma N, Chiaravalli S, Podda M, Casanova M, Gasparini P, Pecori E, Alessandro O, Nigro O, Sironi G, Gattuso G, Terenziani M, Spreafico F, Meazza C, Biassoni V, Schiavello E, Massimino M, Luksch R, Ferrari A
Pediatr Blood Cancer 2022 May;69(5):e29512. Epub 2021 Dec 28 doi: 10.1002/pbc.29512. PMID: 34962687
Outcomes of extraskeletal vs. skeletal Ewing sarcoma patients treated with standard chemotherapy protocol.
Salah S, Abuhijla F, Ismail T, Yaser S, Sultan I, Halalsheh H, Shehadeh A, Abdelal S, Almousa A, Jaber O, Abu-Hijlih R
Clin Transl Oncol 2020 Jun;22(6):878-883. Epub 2019 Aug 19 doi: 10.1007/s12094-019-02202-y. PMID: 31429039
Extraskeletal Ewing's sarcoma: a report of 18 cases and literature review.
Xie CF, Liu MZ, Xi M
Chin J Cancer 2010 Apr;29(4):420-4. doi: 10.5732/cjc.009.10402. PMID: 20346219

Prognosis

Gastrointestinal Ewing Sarcoma: A Clinicopathological and Molecular Genetic Analysis of 25 Cases.
Yin T, Shao M, Sun M, Zhao L, Lao IW, Yu L, Wang J
Am J Surg Pathol 2024 Mar 1;48(3):275-283. Epub 2023 Dec 8 doi: 10.1097/PAS.0000000000002163. PMID: 38062799
Prognostic Factors and Clinical Outcomes in Extraskeletal Ewing Sarcoma: A Cohort Study.
Mathew J, Arjunan R, Dasappa A, Namachivayam A
Ann Surg Oncol 2023 May;30(5):3084-3094. Epub 2022 Dec 23 doi: 10.1245/s10434-022-12992-1. PMID: 36564656
Extraskeletal Ewing Sarcoma from Head to Toe: Multimodality Imaging Review.
Wright A, Desai M, Bolan CW, Badawy M, Guccione J, Rao Korivi B, Pickhardt PJ, Mellnick VM, Lubner MG, Chen L, Elsayes KM
Radiographics 2022 Jul-Aug;42(4):1145-1160. Epub 2022 May 27 doi: 10.1148/rg.210226. PMID: 35622491
Ewing's sarcoma of the thumb.
Yamaguchi T, Tamai K, Saotome K, Hoshino T, Masawa N
Skeletal Radiol 1997 Dec;26(12):725-8. doi: 10.1007/s002560050319. PMID: 9453107
Malignant neuroepithelioma (peripheral neuroblastoma). A clinicopathologic study of 15 cases.
Hashimoto H, Enjoji M, Nakajima T, Kiryu H, Daimaru Y
Am J Surg Pathol 1983 Jun;7(4):309-18. doi: 10.1097/00000478-198306000-00002. PMID: 6869663

Clinical prediction guides

Extraskeletal, intradural, non-metastatic Ewing's sarcoma. Case report.
Ottóffy G, Komáromy H
Ideggyogy Sz 2020 Jul 30;73(7-08):286-288. doi: 10.18071/isz.73.0268. PMID: 32750246
Extraskeletal versus Skeletal Ewing Sarcoma in the adult population: Controversies in care.
Lynch AD, Gani F, Meyer CF, Morris CD, Ahuja N, Johnston FM
Surg Oncol 2018 Sep;27(3):373-379. Epub 2018 May 24 doi: 10.1016/j.suronc.2018.05.016. PMID: 30217290
Treatment outcomes of Japanese patients with Ewing sarcoma: differences between skeletal and extraskeletal Ewing sarcoma.
Takenaka S, Naka N, Obata H, Joyama S, Hamada K, Imura Y, Kakunaga S, Aoki Y, Ueda T, Araki N, Yoshikawa H
Jpn J Clin Oncol 2016 Jun;46(6):522-8. Epub 2016 Mar 23 doi: 10.1093/jjco/hyw032. PMID: 27008849
Extraskeletal Ewing's sarcoma family of tumors in adults: prognostic factors and clinical outcome.
Tural D, Molinas Mandel N, Dervisoglu S, Oner Dincbas F, Koca S, Colpan Oksuz D, Kantarci F, Turna H, Selcukbiricik F, Hiz M
Jpn J Clin Oncol 2012 May;42(5):420-6. Epub 2012 Mar 12 doi: 10.1093/jjco/hys027. PMID: 22416252
Clinical features and outcomes in patients with Ewing sarcoma and regional lymph node involvement.
Applebaum MA, Goldsby R, Neuhaus J, DuBois SG
Pediatr Blood Cancer 2012 Oct;59(4):617-20. Epub 2011 Dec 19 doi: 10.1002/pbc.24053. PMID: 22184129Free PMC Article

Recent systematic reviews

Primary extraskeletal intradural Ewing sarcoma with acute hemorrhage: a case report and review of the literature.
Salama H, Abu-Hilal LH, Idkedek M, Kharousha A, Abulihya M, Nimer H
J Med Case Rep 2024 Mar 9;18(1):144. doi: 10.1186/s13256-024-04384-8. PMID: 38459600Free PMC Article

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