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Ewing sarcoma of bone

MedGen UID:
108438
Concept ID:
C0585474
Neoplastic Process
Synonyms: bone Ewing sarcoma; Bone Ewing's Sarcoma; bone Ewing's sarcoma; bone localised Ewing sarcoma; bone localised Ewing's sarcoma; bone localized Ewing sarcoma; bone localized Ewing's sarcoma; bone tissue Ewing sarcoma; Ewing Sarcoma of Bone; Ewing's Sarcoma of Bone; Ewing's sarcoma of bone; Ewing's Sarcoma, Osseous; Ewing's sarcoma, osseous; Ewing's sarcoma/bone peripheral primitive neuroectodermal tumor; Ewing's sarcoma/bone peripheral primitive neuroectodermal tumour; localised skeletal Ewing's sarcoma; localized skeletal Ewing's sarcoma; Osseous Ewing's Sarcoma; osseous Ewing's sarcoma; Osseous Ewing's Tumor; osseous Ewing's tumor; osseous Ewing's tumour; Skeletal Ewing's Sarcoma; skeletal Ewing's sarcoma; Skeletal Ewing's Tumor; skeletal Ewing's tumor; skeletal Ewing's tumour
SNOMED CT: Ewing sarcoma of bone (307608006); Ewing's sarcoma of bone (307608006)
 
Monarch Initiative: MONDO:0002625

Definition

A small round cell bone tumor that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It represents one of the two ends of the spectrum called Ewing sarcoma/peripheral neuroectodermal tumor. It often affects the diaphysis or metaphyseal-diaphyseal portion of long bones. Clinical findings include pain and a mass in the involved area. Fever, anemia, leukocytosis, and an increased sedimentation rate are often seen. X-ray examination reveals osteolytic lesions. The prognosis depends on the stage, anatomic location, and size of the tumor. [from NCI]

Professional guidelines

PubMed

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
Chaber R, Arthur CJ, Łach K, Raciborska A, Michalak E, Bilska K, Drabko K, Depciuch J, Kaznowska E, Cebulski J
Molecules 2019 Mar 19;24(6) doi: 10.3390/molecules24061075. PMID: 30893786Free PMC Article
Zhang WT, Zhang WW, He ZY, Sun JY, Zhang L, Xia Q, Wu SG
Expert Rev Anticancer Ther 2018 May;18(5):501-506. Epub 2018 Mar 21 doi: 10.1080/14737140.2018.1453360. PMID: 29537323

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Bone Cancer, 2024

Recent clinical studies

Etiology

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
Brennan B, Kirton L, Marec-Bérard P, Gaspar N, Laurence V, Martín-Broto J, Sastre A, Gelderblom H, Owens C, Fenwick N, Strauss S, Moroz V, Whelan J, Wheatley K
Lancet 2022 Oct 29;400(10362):1513-1521. doi: 10.1016/S0140-6736(22)01790-1. PMID: 36522207
Brown JM, Rakoczy K, Tokson JH, Jones KB, Groundland JS
Cancer Treat Res Commun 2022;33:100634. Epub 2022 Sep 13 doi: 10.1016/j.ctarc.2022.100634. PMID: 36126512
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
Donaldson SS
Pediatr Blood Cancer 2004 May;42(5):471-6. doi: 10.1002/pbc.10472. PMID: 15049023

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
Brennan B, Kirton L, Marec-Bérard P, Gaspar N, Laurence V, Martín-Broto J, Sastre A, Gelderblom H, Owens C, Fenwick N, Strauss S, Moroz V, Whelan J, Wheatley K
Lancet 2022 Oct 29;400(10362):1513-1521. doi: 10.1016/S0140-6736(22)01790-1. PMID: 36522207
Brown JM, Rakoczy K, Tokson JH, Jones KB, Groundland JS
Cancer Treat Res Commun 2022;33:100634. Epub 2022 Sep 13 doi: 10.1016/j.ctarc.2022.100634. PMID: 36126512
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
Chaber R, Arthur CJ, Łach K, Raciborska A, Michalak E, Bilska K, Drabko K, Depciuch J, Kaznowska E, Cebulski J
Molecules 2019 Mar 19;24(6) doi: 10.3390/molecules24061075. PMID: 30893786Free PMC Article

Therapy

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
Brennan B, Kirton L, Marec-Bérard P, Gaspar N, Laurence V, Martín-Broto J, Sastre A, Gelderblom H, Owens C, Fenwick N, Strauss S, Moroz V, Whelan J, Wheatley K
Lancet 2022 Oct 29;400(10362):1513-1521. doi: 10.1016/S0140-6736(22)01790-1. PMID: 36522207
Brown JM, Rakoczy K, Tokson JH, Jones KB, Groundland JS
Cancer Treat Res Commun 2022;33:100634. Epub 2022 Sep 13 doi: 10.1016/j.ctarc.2022.100634. PMID: 36126512
Nakano K
Int J Mol Sci 2022 Mar 24;23(7) doi: 10.3390/ijms23073540. PMID: 35408900Free PMC Article
Chaber R, Arthur CJ, Łach K, Raciborska A, Michalak E, Bilska K, Drabko K, Depciuch J, Kaznowska E, Cebulski J
Molecules 2019 Mar 19;24(6) doi: 10.3390/molecules24061075. PMID: 30893786Free PMC Article

Prognosis

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
Brown JM, Rakoczy K, Tokson JH, Jones KB, Groundland JS
Cancer Treat Res Commun 2022;33:100634. Epub 2022 Sep 13 doi: 10.1016/j.ctarc.2022.100634. PMID: 36126512
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
Handl M, Hermanova M, Hotarkova S, Jarkovsky J, Mudry P, Shatokhina T, Vesela M, Sterba J, Zambo I
Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2018 Mar;162(1):54-60. Epub 2017 Nov 21 doi: 10.5507/bp.2017.049. PMID: 29170560
Donaldson SS
Pediatr Blood Cancer 2004 May;42(5):471-6. doi: 10.1002/pbc.10472. PMID: 15049023

Clinical prediction guides

Righi A, Pacheco M, Palmerini E, Carretta E, Gambarotti M, Longhi A, Magagnoli G, Sbaraglia M, Manfrini M, Picci P, Dei Tos AP
Eur J Surg Oncol 2021 Jul;47(7):1778-1783. Epub 2021 Feb 16 doi: 10.1016/j.ejso.2021.02.009. PMID: 33622576
Chaber R, Arthur CJ, Łach K, Raciborska A, Michalak E, Bilska K, Drabko K, Depciuch J, Kaznowska E, Cebulski J
Molecules 2019 Mar 19;24(6) doi: 10.3390/molecules24061075. PMID: 30893786Free PMC Article
DuBois SG, Krailo MD, Gebhardt MC, Donaldson SS, Marcus KJ, Dormans J, Shamberger RC, Sailer S, Nicholas RW, Healey JH, Tarbell NJ, Randall RL, Devidas M, Meyer JS, Granowetter L, Womer RB, Bernstein M, Marina N, Grier HE
Cancer 2015 Feb 1;121(3):467-75. Epub 2014 Sep 23 doi: 10.1002/cncr.29065. PMID: 25251206Free PMC Article
López Guerra JL, Márquez-Vega C, Ramírez-Villar GL, Cabrera P, Ordóñez R, Praena-Fernández JM, Ortiz MJ
Clin Transl Oncol 2012 Apr;14(4):294-301. doi: 10.1007/s12094-012-0798-y. PMID: 22484637
Lemmi MA, Fletcher BD, Marina NM, Slade W, Parham DM, Jenkins JJ, Meyer WH
AJR Am J Roentgenol 1990 Aug;155(2):343-6. doi: 10.2214/ajr.155.2.2115265. PMID: 2115265

Recent systematic reviews

Chen S, Yang L, Pu F, Lin H, Wang B, Liu J, Shao Z
Int J Environ Res Public Health 2015 Sep 9;12(9):11178-95. doi: 10.3390/ijerph120911178. PMID: 26371026Free PMC Article

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Bone Cancer, 2024

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