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Extraskeletal Ewing sarcoma

MedGen UID:
76053
Concept ID:
C0279980
Neoplastic Process
Synonyms: EOE; Extra-Osseous Ewing's Sarcoma; Extra-osseous Ewing's sarcoma; Extraosseous Ewing Sarcoma; Extraosseous Ewing sarcoma; extraosseous Ewing sarcoma; Extraosseous Ewing tumor; extraosseous Ewing tumor; extraosseous Ewing tumour; Extraosseous Ewing's Sarcoma; extraosseous Ewing's sarcoma; extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumour; Extraosseous Ewing's Tumor; extraosseous Ewing's tumor; extraosseous Ewing's tumour; Extraskeletal Ewing Sarcoma; extraskeletal Ewing sarcoma; Extraskeletal Ewing tumor; extraskeletal Ewing tumor; extraskeletal Ewing tumour; Extraskeletal Ewing's Sarcoma; extraskeletal Ewing's sarcoma
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018270
Orphanet: ORPHA370334

Definition

Extraskeletal Ewing sarcoma is a rare, poorly differentiated, highly malignant, soft tissue tumor, derived from neuroectoderm, that is morphologically indistinguishable from skeletal Ewing sarcoma but is located in extraosseous locations, with the most common being: chest wall, paravertebral region, abdominopelvic area (with predilection for the retroperitoneal space), gluteal region and lower extremities. Clinical presentation is highly variable and depends on tumor localization. Local recurrence is common and metastatic disease most frequently involves the bones and lungs. [from ORDO]

Professional guidelines

PubMed

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
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
Thomas DM, O'Sullivan B, Gronchi A
Expert Rev Anticancer Ther 2009 Aug;9(8):1145-57. doi: 10.1586/era.09.77. PMID: 19671034

Recent clinical studies

Etiology

Shiyanbola O, Nigdelioglu R, Dhall D, González IA, Warmke LM, Schechter S, Choi WT, Hu S, Voltaggio L, Zhang Y, Liang TZ, Ko HM, Charville GW, Longacre TA
Am J Surg Pathol 2024 Sep 1;48(9):1185-1194. Epub 2024 May 17 doi: 10.1097/PAS.0000000000002236. PMID: 38767576Free PMC Article
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
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
Kunisada T, Nakata E, Fujiwara T, Hosono A, Takihira S, Kondo H, Ozaki T
Int J Clin Oncol 2023 Jan;28(1):1-11. Epub 2022 Jan 27 doi: 10.1007/s10147-022-02119-7. PMID: 35084598
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

Diagnosis

Shiyanbola O, Nigdelioglu R, Dhall D, González IA, Warmke LM, Schechter S, Choi WT, Hu S, Voltaggio L, Zhang Y, Liang TZ, Ko HM, Charville GW, Longacre TA
Am J Surg Pathol 2024 Sep 1;48(9):1185-1194. Epub 2024 May 17 doi: 10.1097/PAS.0000000000002236. PMID: 38767576Free PMC Article
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
Hassan R, Meng LV, Ngee KT, I-Vern L, Sankaran P, Hean LC, Euxian L, Mohamad H, Dusa NM, Subramaniam M
Lancet 2022 Mar 26;399(10331):1265. doi: 10.1016/S0140-6736(22)00361-0. PMID: 35339226
Wang C, Dickson BC, Smith AC, Hope AJ, Ko HM
Cytopathology 2022 May;33(3):389-392. Epub 2021 Nov 17 doi: 10.1111/cyt.13074. PMID: 34747554
Anik Y, Corapcioglu F, Yildiz DK
Pediatr Hematol Oncol 2008 Jun;25(5):469-71. doi: 10.1080/08880010802104486. PMID: 18569849

Therapy

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
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
Buffoni I, Nuri H, Pome' G, Sementa AR, Stagnaro N, Barra S, Manzitti C, Garaventa A
J Pediatr Hematol Oncol 2021 May 1;43(4):147-151. doi: 10.1097/MPH.0000000000001622. PMID: 31688622
Hopp AC, Nguyen BD
J Gastroenterol Hepatol 2019 Aug;34(8):1273. Epub 2019 Mar 25 doi: 10.1111/jgh.14638. PMID: 30912185
Li T, Goldberg RA, Becker B, McCann J
Arch Ophthalmol 2003 Jul;121(7):1049-52. doi: 10.1001/archopht.121.7.1049. PMID: 12860815

Prognosis

Shiyanbola O, Nigdelioglu R, Dhall D, González IA, Warmke LM, Schechter S, Choi WT, Hu S, Voltaggio L, Zhang Y, Liang TZ, Ko HM, Charville GW, Longacre TA
Am J Surg Pathol 2024 Sep 1;48(9):1185-1194. Epub 2024 May 17 doi: 10.1097/PAS.0000000000002236. PMID: 38767576Free PMC Article
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
Kunisada T, Nakata E, Fujiwara T, Hosono A, Takihira S, Kondo H, Ozaki T
Int J Clin Oncol 2023 Jan;28(1):1-11. Epub 2022 Jan 27 doi: 10.1007/s10147-022-02119-7. PMID: 35084598
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
Ozturk E, Mutlu H, Sonmez G, Vardar Aker F, Cinar Basekim C, Kizilkaya E
J Neuroradiol 2007 Mar;34(1):63-7. doi: 10.1016/j.neurad.2007.01.009. PMID: 17316801

Clinical prediction guides

Shiyanbola O, Nigdelioglu R, Dhall D, González IA, Warmke LM, Schechter S, Choi WT, Hu S, Voltaggio L, Zhang Y, Liang TZ, Ko HM, Charville GW, Longacre TA
Am J Surg Pathol 2024 Sep 1;48(9):1185-1194. Epub 2024 May 17 doi: 10.1097/PAS.0000000000002236. PMID: 38767576Free PMC Article
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
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
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
Thomas DM, O'Sullivan B, Gronchi A
Expert Rev Anticancer Ther 2009 Aug;9(8):1145-57. doi: 10.1586/era.09.77. PMID: 19671034

Recent systematic reviews

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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