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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
Vincristine, irinotecan, and temozolomide treatment for refractory/relapsed pediatric solid tumors: A single center experience.
Büyükkapu Bay S, Kebudi R, Görgün O, Zülfikar B, Darendeliler E, Çakır FB
J Oncol Pharm Pract 2019 Sep;25(6):1343-1348. Epub 2018 Aug 6 doi: 10.1177/1078155218790798. PMID: 30080131
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

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 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 peripheral neuroectodermal tumor (PNET) of the adrenal gland: a rare entity.
Koufopoulos N, Kokkali S, Manatakis D, Balalis D, Nasi D, Ardavanis A, Korkolis D, Khaldi L
J BUON 2019 Mar-Apr;24(2):770-778. PMID: 31128035
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

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 peripheral neuroectodermal tumor (PNET) of the adrenal gland: a rare entity.
Koufopoulos N, Kokkali S, Manatakis D, Balalis D, Nasi D, Ardavanis A, Korkolis D, Khaldi L
J BUON 2019 Mar-Apr;24(2):770-778. PMID: 31128035
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'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

Clinical prediction guides

Membrane-type 1 matrix metalloproteinase as predictor of survival and candidate therapeutic target in Ewing sarcoma.
Brookes MJ, Roundhill EA, Jeys L, Parry M, Burchill SA, Rankin KS
Pediatr Blood Cancer 2022 Dec;69(12):e29959. Epub 2022 Sep 15 doi: 10.1002/pbc.29959. PMID: 36106829
Prognostic value of detection of CD99(+) , CD45(-) cells in peripheral blood by flow cytometry in children with Ewing sarcoma.
Zakzok O, Elshanshory M, Zekri W, Elsharkawy N, Zaky I, Salama A, Kamel A, Elantably I, Said S
Pediatr Blood Cancer 2022 Jan;69(1):e29298. Epub 2021 Aug 17 doi: 10.1002/pbc.29298. PMID: 34403190
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 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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