U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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: 36184748
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
Treatment of children with peripheral primitive neuroectodermal tumor or extraosseous Ewing's tumor with Ewing's-directed therapy.
Gururangan S, Marina NM, Luo X, Parham DM, Tzen CY, Greenwald CA, Rao BN, Kun LE, Meyer WH
J Pediatr Hematol Oncol 1998 Jan-Feb;20(1):55-61. doi: 10.1097/00043426-199801000-00009. PMID: 9482414

Recent clinical studies

Etiology

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
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

Extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumour of the kidney: a case report and literature review.
Li J, Nie F, Li Y
BMC Urol 2022 Nov 30;22(1):197. doi: 10.1186/s12894-022-01146-w. PMID: 36451163Free PMC Article
Primary vulvar Ewing sarcoma/peripheral primitive neuroectodermal tumor with pelvic lymph nodes metastasis: A case report and review of literature.
El Housheimi A, Khalil A, Khalifeh D, Berjawi G, Seoud M, Tabbarah A, Khaled C, Mahfouz R, Abdallah R
J Obstet Gynaecol Res 2020 Oct;46(10):2185-2192. Epub 2020 Aug 5 doi: 10.1111/jog.14399. PMID: 32761741
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

Therapy

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
Primitive neuroectodermal tumor of the cervix uteri: a case report -- changing concepts in therapy.
Snijders-Keilholz A, Ewing P, Seynaeve C, Burger CW
Gynecol Oncol 2005 Sep;98(3):516-9. doi: 10.1016/j.ygyno.2005.05.020. PMID: 15979131
Ewing's sarcoma and peripheral primitive neuroectodermal tumor.
Jürgens HF
Curr Opin Oncol 1994 Jul;6(4):391-6. PMID: 7803540
Autologous bone marrow transplantation in paediatric solid tumours.
Pinkerton R, Philip T, Bouffet E, Lashford L, Kemshead J
Clin Haematol 1986 Feb;15(1):187-203. doi: 10.1016/s0308-2261(86)80011-x. PMID: 3516489

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
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
Ewing's sarcoma and peripheral primitive neuroectodermal tumor.
Jürgens HF
Curr Opin Oncol 1994 Jul;6(4):391-6. PMID: 7803540

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
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
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

Supplemental Content

Table of contents

    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.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...