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Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

MedGen UID:
167155
•Concept ID:
C0796547
•
Neoplastic Process
Synonyms: Localized Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Localized Tumors of Ewing's Family; Localized Tumors of the Ewing's Family

Definition

A small round cell tumor with or without neural differentiation that is confined to a specific site without evidence of spread to other anatomic sites. [from NCI]

Professional guidelines

PubMed

Irinotecan dose schedule for the treatment of Ewing sarcoma.
Slotkin EK, Meyers PA
Pediatr Blood Cancer 2023 Jan;70(1):e30005. Epub 2022 Oct 2 doi: 10.1002/pbc.30005. PMID: 36184748Free PMC Article
Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.
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
High-dose melphalan therapy for the treatment of children with refractory neuroblastoma and Ewing's sarcoma.
Graham-Pole J, Lazarus HM, Herzig RH, Gross S, Coccia P, Weiner R, Strandjord S
Am J Pediatr Hematol Oncol 1984 Spring;6(1):17-26. PMID: 6370012

Recent clinical studies

Etiology

Children's Oncology Group's 2023 blueprint for research: Bone tumors.
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
Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.
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
Multiple Ewing Sarcoma/Primitive Neuroectodermal Tumors in the Mediastinum: A Case Report and Literature Review.
Bae SH, Hwang JH, Da Nam B, Kim HJ, Kim KU, Kim DW, Choi IH
Medicine (Baltimore) 2016 Feb;95(7):e2725. doi: 10.1097/MD.0000000000002725. PMID: 26886614Free PMC Article
Ewing sarcoma/primitive neuroectodermal tumor of the kidney: clinicopathologic analysis of 34 cases.
Karpate A, Menon S, Basak R, Yuvaraja TB, Tongaonkar HB, Desai SB
Ann Diagn Pathol 2012 Aug;16(4):267-74. Epub 2012 Apr 23 doi: 10.1016/j.anndiagpath.2011.07.011. PMID: 22534242
Ewing's sarcoma and peripheral primitive neuroectodermal tumors after their genetic union.
Kovar H
Curr Opin Oncol 1998 Jul;10(4):334-42. doi: 10.1097/00001622-199807000-00010. PMID: 9702401

Diagnosis

Children's Oncology Group's 2023 blueprint for research: Bone tumors.
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
Neuropeptide Y Peptide Family and Cancer: Antitumor Therapeutic Strategies.
Sánchez ML, Rodríguez FD, Coveñas R
Int J Mol Sci 2023 Jun 9;24(12) doi: 10.3390/ijms24129962. PMID: 37373115Free PMC Article
Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.
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
Ewing's sarcoma/primitive neuroectodermal tumor arising from the adrenal gland: a case report and literature review.
Sasaki T, Onishi T, Yabana T, Hoshina A
Tumori 2013 May-Jun;99(3):e104-6. doi: 10.1177/030089161309900326. PMID: 24158076
Ewing's sarcoma and peripheral primitive neuroectodermal tumor.
Jürgens HF
Curr Opin Oncol 1994 Jul;6(4):391-6. PMID: 7803540

Therapy

Children's Oncology Group's 2023 blueprint for research: Bone tumors.
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
Irinotecan dose schedule for the treatment of Ewing sarcoma.
Slotkin EK, Meyers PA
Pediatr Blood Cancer 2023 Jan;70(1):e30005. Epub 2022 Oct 2 doi: 10.1002/pbc.30005. PMID: 36184748Free PMC Article
Ewing sarcoma of the adrenal gland: a case report and review of the literature.
Eddaoualline H, Mazouz K, Rafiq B, El Mghari Tabib G, El Ansari N, Belbaraka R, El Omrani A, Khouchani M
J Med Case Rep 2018 Mar 16;12(1):69. doi: 10.1186/s13256-018-1601-7. PMID: 29544549Free PMC Article
Ewing's sarcoma/primitive neuroectodermal tumor arising from the adrenal gland: a case report and literature review.
Sasaki T, Onishi T, Yabana T, Hoshina A
Tumori 2013 May-Jun;99(3):e104-6. doi: 10.1177/030089161309900326. PMID: 24158076
Ewing's sarcoma and peripheral primitive neuroectodermal tumor.
Jürgens HF
Curr Opin Oncol 1994 Jul;6(4):391-6. PMID: 7803540

Prognosis

Primary intracranial Ewing sarcoma/ peripheral primitive neuroectodermal tumor, an entity of unacquaintance: a series of 8 cases.
Deshpande G, Epari S, Gupta C, Shetty O, Gurav M, Chinnaswamy G, Moiyadi A, Gupta T
Childs Nerv Syst 2021 Mar;37(3):839-849. Epub 2020 Aug 6 doi: 10.1007/s00381-020-04850-w. PMID: 32761378
Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.
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
Malignant osseous tumors of the pediatric spine.
Kim HJ, McLawhorn AS, Goldstein MJ, Boland PJ
J Am Acad Orthop Surg 2012 Oct;20(10):646-56. doi: 10.5435/JAAOS-20-10-646. PMID: 23027694
Ewing sarcoma/primitive neuroectodermal tumor of the kidney: clinicopathologic analysis of 34 cases.
Karpate A, Menon S, Basak R, Yuvaraja TB, Tongaonkar HB, Desai SB
Ann Diagn Pathol 2012 Aug;16(4):267-74. Epub 2012 Apr 23 doi: 10.1016/j.anndiagpath.2011.07.011. PMID: 22534242
Ewing's sarcoma and peripheral primitive neuroectodermal tumor.
Jürgens HF
Curr Opin Oncol 1994 Jul;6(4):391-6. PMID: 7803540

Clinical prediction guides

Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.
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
Ewing's sarcoma/primitive neuroectodermal tumor arising from the adrenal gland: a case report and literature review.
Sasaki T, Onishi T, Yabana T, Hoshina A
Tumori 2013 May-Jun;99(3):e104-6. doi: 10.1177/030089161309900326. PMID: 24158076
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
Adult soft tissue Ewing sarcoma or primitive neuroectodermal tumors: predictors of survival?
Martin RC 2nd, Brennan MF
Arch Surg 2003 Mar;138(3):281-5. doi: 10.1001/archsurg.138.3.281. PMID: 12611575
Ewing's sarcoma and peripheral primitive neuroectodermal tumors after their genetic union.
Kovar H
Curr Opin Oncol 1998 Jul;10(4):334-42. doi: 10.1097/00001622-199807000-00010. PMID: 9702401

Recent systematic reviews

(18) F-FDG PET and PET/CT as a diagnostic method for Ewing sarcoma: A systematic review and meta-analysis.
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
Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.
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

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