U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Ewing sarcoma/peripheral primitive neuroectodermal tumor of bone

MedGen UID:
272598
Concept ID:
C1333481
Neoplastic Process
Synonyms: bone tissue Ewing sarcoma/peripheral primitive neuroectodermal tumor; bone tissue Ewing sarcoma/peripheral primitive neuroectodermal tumour; Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor of Bone; Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor of Bone; Ewing's sarcoma/peripheral primitive neuroectodermal tumor of bone; Ewing's sarcoma/peripheral primitive neuroectodermal tumour of bone
 
Monarch Initiative: MONDO:0021123

Definition

A spectrum of malignant tumors arising from the bone and characterized morphologically by the presence of small round cells. Ewing sarcoma and peripheral primitive neuroectodermal tumor represent the ends of a spectrum, with Ewing sarcoma lacking evidence of neural differentiation and the markers that characterize the peripheral primitive neuroectodermal tumor. Ewing sarcoma and peripheral primitive neuroectodermal tumor may share cytogenetic abnormalities, proto-oncogene expression, cell culture and immunohistochemical abnormalities. Pain and the presence of a mass are the most common clinical symptoms. [from NCI]

Professional guidelines

PubMed

Breneman JC, Donaldson SS, Constine L, Merchant T, Marcus K, Paulino AC, Followill D, Mahajan A, Laack N, Esiashvili N, Haas-Kogan D, Laurie F, Olch A, Ulin K, Hodgson D, Yock TI, Terezakis S, Krasin M, Panoff J, Chuba P, Hua CH, Hess CB, Houghton PJ, Wolden S, Buchsbaum J, Fitzgerald TJ, Kalapurakal JA
Int J Radiat Oncol Biol Phys 2018 Jul 15;101(4):860-874. Epub 2018 Mar 14 doi: 10.1016/j.ijrobp.2018.03.002. PMID: 29976498Free PMC Article
Cherif El Asri A, Benzagmout M, Chakour K, Chaoui MF, Laaguili J, Chahdi H, Gazzaz M, El Mostarchid B
World Neurosurg 2018 Jul;115:346-356. Epub 2018 May 3 doi: 10.1016/j.wneu.2018.04.164. PMID: 29729469
Tsai EC, Santoreneos S, Rutka JT
Neurosurg Focus 2002 May 15;12(5):e1. doi: 10.3171/foc.2002.12.5.2. PMID: 16119897

Recent clinical studies

Etiology

Zhou H, He S, Zhang D, Wang J, Yang X, Jiao J, Xu W, Yang J, Xiao J
Medicine (Baltimore) 2023 Oct 27;102(43):e34231. doi: 10.1097/MD.0000000000034231. PMID: 37904412Free PMC Article
Reed DR, Grohar P, Rubin E, Binitie O, Krailo M, Davis J, DuBois SG, Janeway KA
Pediatr Blood Cancer 2023 Sep;70 Suppl 6(Suppl 6):e30583. Epub 2023 Jul 27 doi: 10.1002/pbc.30583. PMID: 37501549Free PMC Article
Chen J, Wu SZ, Tan J, Zhang QY, Qin BQ, Wang Y, Zhang H
BMC Surg 2022 Jan 8;22(1):4. doi: 10.1186/s12893-021-01452-4. PMID: 34996420Free PMC Article
Mobley BC, Roulston D, Shah GV, Bijwaard KE, McKeever PE
Hum Pathol 2006 Jul;37(7):845-53. Epub 2006 May 19 doi: 10.1016/j.humpath.2006.02.011. PMID: 16784984
de Alava E, Pardo J
Int J Surg Pathol 2001 Jan;9(1):7-17. doi: 10.1177/106689690100900104. PMID: 11469351

Diagnosis

Kantiwal P, Yadav SK, Nalwa A, Rajnish RK
BMJ Case Rep 2023 Dec 7;16(12) doi: 10.1136/bcr-2023-258243. PMID: 38061850Free PMC Article
Patra S, Trivedi P
Malays J Pathol 2022 Apr;44(1):93-99. PMID: 35484891
Cherif El Asri A, Benzagmout M, Chakour K, Chaoui MF, Laaguili J, Chahdi H, Gazzaz M, El Mostarchid B
World Neurosurg 2018 Jul;115:346-356. Epub 2018 May 3 doi: 10.1016/j.wneu.2018.04.164. PMID: 29729469
Mobley BC, Roulston D, Shah GV, Bijwaard KE, McKeever PE
Hum Pathol 2006 Jul;37(7):845-53. Epub 2006 May 19 doi: 10.1016/j.humpath.2006.02.011. PMID: 16784984
de Alava E, Pardo J
Int J Surg Pathol 2001 Jan;9(1):7-17. doi: 10.1177/106689690100900104. PMID: 11469351

Therapy

Reed DR, Grohar P, Rubin E, Binitie O, Krailo M, Davis J, DuBois SG, Janeway KA
Pediatr Blood Cancer 2023 Sep;70 Suppl 6(Suppl 6):e30583. Epub 2023 Jul 27 doi: 10.1002/pbc.30583. PMID: 37501549Free PMC Article
Li Q, Liu Y, Yu Y
Medicine (Baltimore) 2017 Dec;96(51):e9272. doi: 10.1097/MD.0000000000009272. PMID: 29390485Free PMC Article
Carvajal R, Meyers P
Hematol Oncol Clin North Am 2005 Jun;19(3):501-25, vi-vii. doi: 10.1016/j.hoc.2005.03.004. PMID: 15939194
Kovar H, Aryee D, Zoubek A
Curr Opin Oncol 1999 Jul;11(4):275-84. doi: 10.1097/00001622-199907000-00007. PMID: 10416880
Jürgens HF
Curr Opin Oncol 1994 Jul;6(4):391-6. PMID: 7803540

Prognosis

Thakur MD, Franz CJ, Brennan L, Brouwer-Visser J, Tam R, Korski K, Koeppen H, Ziai J, Babitzki G, Ranchere-Vince D, Vasiljevic A, Dijoud F, Marec-Bérard P, Rochet I, Cannarile MA, Marabelle A
Eur J Cancer 2022 Jul;170:179-193. Epub 2022 May 31 doi: 10.1016/j.ejca.2022.03.012. PMID: 35660252
Patra S, Trivedi P
Malays J Pathol 2022 Apr;44(1):93-99. PMID: 35484891
Celli R, Cai G
Arch Pathol Lab Med 2016 Mar;140(3):281-5. doi: 10.5858/arpa.2014-0367-RS. PMID: 26927724
Jin SG, Jiang XP, Zhong L
Pediatr Neonatol 2016 Oct;57(5):436-439. Epub 2014 Jan 27 doi: 10.1016/j.pedneo.2013.11.002. PMID: 24480101
de Alava E, Pardo J
Int J Surg Pathol 2001 Jan;9(1):7-17. doi: 10.1177/106689690100900104. PMID: 11469351

Clinical prediction guides

Liu L, Li L, Ding Y, Kong F, Mo W, Ye H, Shen D
Diagn Pathol 2024 Jan 22;19(1):19. doi: 10.1186/s13000-024-01443-y. PMID: 38254207Free PMC Article
Zhou H, He S, Zhang D, Wang J, Yang X, Jiao J, Xu W, Yang J, Xiao J
Medicine (Baltimore) 2023 Oct 27;102(43):e34231. doi: 10.1097/MD.0000000000034231. PMID: 37904412Free PMC Article
Cherif El Asri A, Benzagmout M, Chakour K, Chaoui MF, Laaguili J, Chahdi H, Gazzaz M, El Mostarchid B
World Neurosurg 2018 Jul;115:346-356. Epub 2018 May 3 doi: 10.1016/j.wneu.2018.04.164. PMID: 29729469
Ba L, Tan H, Xiao H, Guan Y, Gao J, Gao X
Acta Radiol 2015 Jul;56(7):820-8. Epub 2014 Jul 29 doi: 10.1177/0284185114539321. PMID: 25073463
Carvajal R, Meyers P
Hematol Oncol Clin North Am 2005 Jun;19(3):501-25, vi-vii. doi: 10.1016/j.hoc.2005.03.004. PMID: 15939194

Recent systematic reviews

Seth N, Seth I, Bulloch G, Siu AHY, Guo A, Chatterjee R, MacManus M, Donnan L
Pediatr Blood Cancer 2022 Mar;69(3):e29415. Epub 2021 Oct 28 doi: 10.1002/pbc.29415. PMID: 34709700
Cherif El Asri A, Benzagmout M, Chakour K, Chaoui MF, Laaguili J, Chahdi H, Gazzaz M, El Mostarchid B
World Neurosurg 2018 Jul;115:346-356. Epub 2018 May 3 doi: 10.1016/j.wneu.2018.04.164. PMID: 29729469

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...