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Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

MedGen UID:
277363
•Concept ID:
C1334718
•
Neoplastic Process
Synonym: Metastatic Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor

Definition

A small round cell tumor with or without neural differentiation that has spread from its original site of growth to another anatomic site. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMetastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

Professional guidelines

PubMed

Targeting B7-H3-A Novel Strategy for the Design of Anticancer Agents for Extracranial Pediatric Solid Tumors Treatment.
Rasic P, Jeremic M, Jeremic R, Dusanovic Pjevic M, Rasic M, Djuricic SM, Milickovic M, Vukadin M, Mijovic T, Savic D
Molecules 2023 Apr 11;28(8) doi: 10.3390/molecules28083356. PMID: 37110590Free PMC Article
Chemotherapy as an alternative to laminectomy and radiation in the management of epidural tumor.
Hayes FA, Thompson EI, Hvizdala E, O'Connor D, Green AA
J Pediatr 1984 Feb;104(2):221-4. doi: 10.1016/s0022-3476(84)80996-8. PMID: 6694014
Neuroblastoma and the differential diagnosis of small-, round-, blue-cell tumors.
Triche TJ, Askin FB
Hum Pathol 1983 Jul;14(7):569-95. doi: 10.1016/s0046-8177(83)80202-0. PMID: 6345338

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
Feasibility of indocyanine green-guided localization of pulmonary nodules in children with solid tumors.
Abdelhafeez AH, Mothi SS, Pio L, Mori M, Santiago TC, McCarville MB, Kaste SC, Pappo AS, Talbot LJ, Murphy AJ, Davidoff AM
Pediatr Blood Cancer 2023 Oct;70(10):e30437. Epub 2023 May 17 doi: 10.1002/pbc.30437. PMID: 37194488Free PMC Article
Prospective Clinical Genomic Profiling of Ewing Sarcoma: ERF and FGFR1 Mutations as Recurrent Secondary Alterations of Potential Biologic and Therapeutic Relevance.
Ogura K, Elkrief A, Bowman AS, Koche RP, de Stanchina E, Benayed R, Mauguen A, Mattar MS, Khodos I, Meyers PA, Healey JH, Tap WD, Hameed M, Zehir A, Shukla N, Sawyers C, Bose R, Slotkin E, Ladanyi M
JCO Precis Oncol 2022 Aug;6:e2200048. doi: 10.1200/PO.22.00048. PMID: 35952322Free PMC Article
Small cell tumors of bone.
Li S, Siegal GP
Adv Anat Pathol 2010 Jan;17(1):1-11. doi: 10.1097/PAP.0b013e3181bb6b9c. PMID: 20032633
Ewing tumor: tumor biology and clinical applications.
de Alava E, Pardo J
Int J Surg Pathol 2001 Jan;9(1):7-17. doi: 10.1177/106689690100900104. PMID: 11469351

Diagnosis

Non-chemotherapy adjuvant agents in TP53 mutant Ewing sarcoma.
Kim JA, Crawford KA, Spada PA, Martin LR, Zhang J, Wong R, Reid JM, Stewart CF, Frank TM, Liu Q, Michalek JE, Keller C
Sci Rep 2023 Sep 1;13(1):14360. doi: 10.1038/s41598-023-40751-z. PMID: 37658148Free PMC Article
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 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
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor in the Adrenal Gland of a Child.
Goudarzipour K, Farahmandi F, Mohammadi A, Taherian R
Iran J Kidney Dis 2018 May;12(3):190-192. PMID: 29891750
Ewing tumor: tumor biology and clinical applications.
de Alava E, Pardo J
Int J Surg Pathol 2001 Jan;9(1):7-17. doi: 10.1177/106689690100900104. PMID: 11469351

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
Targeting B7-H3-A Novel Strategy for the Design of Anticancer Agents for Extracranial Pediatric Solid Tumors Treatment.
Rasic P, Jeremic M, Jeremic R, Dusanovic Pjevic M, Rasic M, Djuricic SM, Milickovic M, Vukadin M, Mijovic T, Savic D
Molecules 2023 Apr 11;28(8) doi: 10.3390/molecules28083356. PMID: 37110590Free PMC Article
Prospective Clinical Genomic Profiling of Ewing Sarcoma: ERF and FGFR1 Mutations as Recurrent Secondary Alterations of Potential Biologic and Therapeutic Relevance.
Ogura K, Elkrief A, Bowman AS, Koche RP, de Stanchina E, Benayed R, Mauguen A, Mattar MS, Khodos I, Meyers PA, Healey JH, Tap WD, Hameed M, Zehir A, Shukla N, Sawyers C, Bose R, Slotkin E, Ladanyi M
JCO Precis Oncol 2022 Aug;6:e2200048. doi: 10.1200/PO.22.00048. PMID: 35952322Free PMC Article
A Phase I Trial of TB-403 in Relapsed Medulloblastoma, Neuroblastoma, Ewing Sarcoma, and Alveolar Rhabdomyosarcoma.
Saulnier-Sholler G, Duda DG, Bergendahl G, Ebb D, Snuderl M, Laetsch TW, Michlitsch J, Hanson D, Isakoff MS, Bielamowicz K, Kraveka JM, Ferguson W, Carmeliet P, De Deene A, Gijsen L, Jain RK
Clin Cancer Res 2022 Sep 15;28(18):3950-3957. doi: 10.1158/1078-0432.CCR-22-1169. PMID: 35833850Free PMC Article
Autotransplantation in solid tumors.
Spitzer G
Blood Rev 1991 Jun;5(2):105-11. doi: 10.1016/0268-960x(91)90042-b. PMID: 1912755

Prognosis

Histone Methyltransferases G9a/Ehmt2 and GLP/Ehmt1 Are Associated with Cell Viability and Poorer Prognosis in Neuroblastoma and Ewing Sarcoma.
Souza BK, Freire NH, Monteiro TS, Herlinger AL, Jaeger M, Dalmolin MGS, de Farias CB, Gregianin L, Brunetto AT, Brunetto AL, Thiele CJ, Roesler R
Int J Mol Sci 2023 Oct 17;24(20) doi: 10.3390/ijms242015242. PMID: 37894922Free 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
Extraosseous Ewing sarcoma and peripheral primitive neuroectodermal tumor of the thyroid gland: Case report and review.
Chirila M, Muresan M, Ciuleanu E, Cosgarea M
Ear Nose Throat J 2013 Apr-May;92(4-5):E3-6. doi: 10.1177/014556131309200419. PMID: 23599116
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 tumor: tumor biology and clinical applications.
de Alava E, Pardo J
Int J Surg Pathol 2001 Jan;9(1):7-17. doi: 10.1177/106689690100900104. PMID: 11469351

Clinical prediction guides

Immature platelet fraction predicts early marrow recovery after severe chemotherapy associated neutropenia.
Salvador C, Meryk A, Hetzer B, Bargehr C, Kropshofer G, Meister B, Anliker M, Crazzolara R
Sci Rep 2023 Feb 27;13(1):3371. doi: 10.1038/s41598-023-30469-3. PMID: 36849723Free PMC Article
A Phase I Trial of TB-403 in Relapsed Medulloblastoma, Neuroblastoma, Ewing Sarcoma, and Alveolar Rhabdomyosarcoma.
Saulnier-Sholler G, Duda DG, Bergendahl G, Ebb D, Snuderl M, Laetsch TW, Michlitsch J, Hanson D, Isakoff MS, Bielamowicz K, Kraveka JM, Ferguson W, Carmeliet P, De Deene A, Gijsen L, Jain RK
Clin Cancer Res 2022 Sep 15;28(18):3950-3957. doi: 10.1158/1078-0432.CCR-22-1169. PMID: 35833850Free PMC Article
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
Cytogenetic analysis in the examination of solid tumors in children.
Mertens F, Mandahl N, Mitelman F, Heim S
Pediatr Hematol Oncol 1994 Jul-Aug;11(4):361-77. doi: 10.3109/08880019409140536. PMID: 7947009

Recent systematic reviews

Primary and metastatic primitive neuroectodermal tumor of the heart: A systematic review.
Hajsadeghi S, Iranpour A, Amouei E, Tehrani NH, Ghavidel A, Ziaie H, Asadi O, Moradians V, Tayebi A, Mestres CA, Manqoutaei SA
Asian Cardiovasc Thorac Ann 2022 Sep;30(7):761-771. Epub 2022 Mar 21 doi: 10.1177/02184923221083434. PMID: 35306869
(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

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