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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

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
Tao HT, Hu Y, Wang JL, Cheng Y, Zhang X, Wang H, Zhang SJ
Asian Pac J Cancer Prev 2013;14(5):2967-71. doi: 10.7314/apjcp.2013.14.5.2967. PMID: 23803063

Diagnosis

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
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
Tang S, Dodd LG
Diagn Cytopathol 2018 Nov;46(11):958-963. Epub 2018 Oct 24 doi: 10.1002/dc.24027. PMID: 30353686
Avcu S, Ozcan HN, Izmirli M, Lemmerling M
Ir J Med Sci 2012 Mar;181(1):123-5. Epub 2010 Jul 30 doi: 10.1007/s11845-010-0534-y. PMID: 20676795
Guiter GE, Gamboni MM, Zakowski MF
Cancer 1999 Jun 25;87(3):141-8. PMID: 10385445

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
Sparber-Sauer M, Ferrari A, Kosztyla D, Ladenstein R, Cecchetto G, Kazanowska B, Scarzello G, Ljungman G, Milano GM, Niggli F, Alaggio R, Vokuhl C, Casanova M, Klingebiel T, Zin A, Koscielniak E, Bisogno G
Pediatr Blood Cancer 2022 Sep;69(9):e29691. Epub 2022 Apr 19 doi: 10.1002/pbc.29691. PMID: 35441463
Koscielniak E, Sparber-Sauer M, Scheer M, Vokuhl C, Kazanowska B, Ladenstein R, Niggli F, Ljungman G, Paulussen M, Bielack SS, Seitz G, Fuchs J, Hallmen E, Klingebiel T, On Behalf Of The Cws Study Group
Pediatr Blood Cancer 2021 Oct;68(10):e29145. Epub 2021 Jun 5 doi: 10.1002/pbc.29145. PMID: 34089219
Klufas MA, Wolden SL, Bohle GC 3rd, Wexler LH, Abramson DH
Ophthalmic Plast Reconstr Surg 2015 Jul-Aug;31(4):e89-91. doi: 10.1097/IOP.0000000000000111. PMID: 24814278Free PMC Article

Prognosis

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
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
Tao HT, Hu Y, Wang JL, Cheng Y, Zhang X, Wang H, Zhang SJ
Asian Pac J Cancer Prev 2013;14(5):2967-71. doi: 10.7314/apjcp.2013.14.5.2967. PMID: 23803063
Koscielniak E, Morgan M, Treuner J
Paediatr Drugs 2002;4(1):21-8. doi: 10.2165/00128072-200204010-00003. PMID: 11817983

Clinical prediction guides

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
Nakanishi K, Kobayashi M, Nakaguchi K, Kyakuno M, Hashimoto N, Onishi H, Maeda N, Nakata S, Kuwabara M, Murakami T, Nakamura H
Magn Reson Med Sci 2007;6(3):147-55. doi: 10.2463/mrms.6.147. PMID: 18037795

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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