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Primitive neuroectodermal tumor

MedGen UID:
64627
•Concept ID:
C0206663
•
Neoplastic Process
Synonyms: Neoplasm, Primitive Neuroepithelial; Neoplasms, Primitive Neuroepithelial; Neuroectodermal Tumor, Primitive; Neuroectodermal Tumors, Primitive; Neuroepithelial Neoplasm, Primitive; Neuroepithelial Neoplasms, Primitive; Neuroepithelial Tumor, Primitive; Neuroepithelial Tumors, Primitive; PNET; PNETs; Primitive Neuroectodermal Tumor; Primitive Neuroectodermal Tumors; Primitive Neuroepithelial Neoplasm; Primitive Neuroepithelial Neoplasms; Primitive Neuroepithelial Tumor; Primitive Neuroepithelial Tumors; Tumor, Primitive Neuroectodermal; Tumor, Primitive Neuroepithelial; Tumors, Primitive Neuroectodermal; Tumors, Primitive Neuroepithelial
SNOMED CT: PNET - Primitive neuroectodermal tumor (39781001); PNET (39781001); Primitive neuroectodermal tumor (699028006); Primitive neuroectodermal tumor (39781001); Neuroepithelioma (55045006)
 
HPO: HP:0030065
Monarch Initiative: MONDO:0005462

Definition

A tumor that originates in cells from the primitive neural crest. This group of tumors is characteirzed by the presence of primitive cells with elements of neuronal and/or glial differentiation. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Primitive neuroectodermal tumor
Follow this link to review classifications for Primitive neuroectodermal tumor in Orphanet.

Professional guidelines

PubMed

DICER1-associated Tumors in the Female Genital Tract: Molecular Basis, Clinicopathologic Features, and Differential Diagnosis.
Han LM, Weiel JJ, Longacre TA, Folkins AK
Adv Anat Pathol 2022 Sep 1;29(5):297-308. Epub 2022 Jul 1 doi: 10.1097/PAP.0000000000000351. PMID: 35778792
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: standard and experimental treatment options.
Subbiah V, Anderson P, Lazar AJ, Burdett E, Raymond K, Ludwig JA
Curr Treat Options Oncol 2009 Apr;10(1-2):126-40. Epub 2009 Jun 17 doi: 10.1007/s11864-009-0104-6. PMID: 19533369

Recent clinical studies

Etiology

The 2022 revision of the World Health Organization classification of tumors of the urinary system and male genital organs: advances and challenges.
Mohanty SK, Lobo A, Cheng L
Hum Pathol 2023 Jun;136:123-143. Epub 2022 Sep 6 doi: 10.1016/j.humpath.2022.08.006. PMID: 36084769
Radiation Therapy Dose Escalation in Unresectable Ewing Sarcoma: Final Results of a Phase 3 Randomized Controlled Trial.
Laskar S, Sinha S, Chatterjee A, Khanna N, Manjali JJ, Puri A, Gulia A, Nayak P, Vora T, Chinnaswamy G, Prasad M, Bajpai J, Juvekar S, Desai S, Janu A, Rangarajan V, Purandare N, Shah S, Rekhi B, Jambhekar N, Muckaden MA, Kurkure P
Int J Radiat Oncol Biol Phys 2022 Aug 1;113(5):996-1002. Epub 2022 May 11 doi: 10.1016/j.ijrobp.2022.04.024. PMID: 35568246
Renal Primitive Neuroectodermal Tumor With Inferior Vena Cava Thrombus: Case Series and Literature Review of a Rare but Challenging Entity.
Xu C, Sun Z, Xiao R, He W, Ma L
Clin Genitourin Cancer 2021 Oct;19(5):e273-e279. Epub 2021 Apr 3 doi: 10.1016/j.clgc.2021.03.017. PMID: 33941490
Temozolomide with irinotecan versus temozolomide, irinotecan plus bevacizumab for recurrent medulloblastoma of childhood: Report of a COG randomized Phase II screening trial.
Levy AS, Krailo M, Chi S, Villaluna D, Springer L, Williams-Hughes C, Fouladi M, Gajjar A
Pediatr Blood Cancer 2021 Aug;68(8):e29031. Epub 2021 Apr 12 doi: 10.1002/pbc.29031. PMID: 33844469Free PMC Article
Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone.
Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, Donaldson SS, Moore S, Rausen AR, Vietti TJ, Miser JS
N Engl J Med 2003 Feb 20;348(8):694-701. doi: 10.1056/NEJMoa020890. PMID: 12594313

Diagnosis

Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature.
Chao X, Bi Y, Li L
Orphanet J Rare Dis 2019 Jun 19;14(1):147. doi: 10.1186/s13023-019-1106-5. PMID: 31217017Free PMC Article
Ewing sarcoma.
Choi EY, Gardner JM, Lucas DR, McHugh JB, Patel RM
Semin Diagn Pathol 2014 Jan;31(1):39-47. Epub 2014 Jan 5 doi: 10.1053/j.semdp.2014.01.002. PMID: 24680181
Renal primitive neuroectodermal tumors.
Bartholow T, Parwani A
Arch Pathol Lab Med 2012 Jun;136(6):686-90. doi: 10.5858/arpa.2011-0104-RS. PMID: 22646279
Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone.
Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, Donaldson SS, Moore S, Rausen AR, Vietti TJ, Miser JS
N Engl J Med 2003 Feb 20;348(8):694-701. doi: 10.1056/NEJMoa020890. PMID: 12594313
Ewing's sarcoma.
Granowetter L
Curr Opin Oncol 1991 Aug;3(4):684-8. PMID: 1932228

Therapy

Radiation Therapy Dose Escalation in Unresectable Ewing Sarcoma: Final Results of a Phase 3 Randomized Controlled Trial.
Laskar S, Sinha S, Chatterjee A, Khanna N, Manjali JJ, Puri A, Gulia A, Nayak P, Vora T, Chinnaswamy G, Prasad M, Bajpai J, Juvekar S, Desai S, Janu A, Rangarajan V, Purandare N, Shah S, Rekhi B, Jambhekar N, Muckaden MA, Kurkure P
Int J Radiat Oncol Biol Phys 2022 Aug 1;113(5):996-1002. Epub 2022 May 11 doi: 10.1016/j.ijrobp.2022.04.024. PMID: 35568246
Temozolomide with irinotecan versus temozolomide, irinotecan plus bevacizumab for recurrent medulloblastoma of childhood: Report of a COG randomized Phase II screening trial.
Levy AS, Krailo M, Chi S, Villaluna D, Springer L, Williams-Hughes C, Fouladi M, Gajjar A
Pediatr Blood Cancer 2021 Aug;68(8):e29031. Epub 2021 Apr 12 doi: 10.1002/pbc.29031. PMID: 33844469Free PMC Article
Human Epidermal Growth Factor Receptor 2 (HER2) -Specific Chimeric Antigen Receptor-Modified T Cells for the Immunotherapy of HER2-Positive Sarcoma.
Ahmed N, Brawley VS, Hegde M, Robertson C, Ghazi A, Gerken C, Liu E, Dakhova O, Ashoori A, Corder A, Gray T, Wu MF, Liu H, Hicks J, Rainusso N, Dotti G, Mei Z, Grilley B, Gee A, Rooney CM, Brenner MK, Heslop HE, Wels WS, Wang LL, Anderson P, Gottschalk S
J Clin Oncol 2015 May 20;33(15):1688-96. Epub 2015 Mar 23 doi: 10.1200/JCO.2014.58.0225. PMID: 25800760Free PMC Article
Renal Ewing tumors.
Zöllner S, Dirksen U, Jürgens H, Ranft A
Ann Oncol 2013 Sep;24(9):2455-61. Epub 2013 Jun 11 doi: 10.1093/annonc/mdt215. PMID: 23761687
Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone.
Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, Donaldson SS, Moore S, Rausen AR, Vietti TJ, Miser JS
N Engl J Med 2003 Feb 20;348(8):694-701. doi: 10.1056/NEJMoa020890. PMID: 12594313

Prognosis

Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature.
Chao X, Bi Y, Li L
Orphanet J Rare Dis 2019 Jun 19;14(1):147. doi: 10.1186/s13023-019-1106-5. PMID: 31217017Free PMC Article
Ewing sarcoma.
Choi EY, Gardner JM, Lucas DR, McHugh JB, Patel RM
Semin Diagn Pathol 2014 Jan;31(1):39-47. Epub 2014 Jan 5 doi: 10.1053/j.semdp.2014.01.002. PMID: 24680181
Primary pericardial tumors.
Restrepo CS, Vargas D, Ocazionez D, Martínez-Jiménez S, Betancourt Cuellar SL, Gutierrez FR
Radiographics 2013 Oct;33(6):1613-30. doi: 10.1148/rg.336135512. PMID: 24108554
Ewing sarcoma/peripheral primitive neuroectodermal tumor and related tumors.
Tsokos M, Alaggio RD, Dehner LP, Dickman PS
Pediatr Dev Pathol 2012;15(1 Suppl):108-26. doi: 10.2350/11-08-1078-PB.1. PMID: 22420726Free PMC Article
Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone.
Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, Donaldson SS, Moore S, Rausen AR, Vietti TJ, Miser JS
N Engl J Med 2003 Feb 20;348(8):694-701. doi: 10.1056/NEJMoa020890. PMID: 12594313

Clinical prediction guides

Radiation Therapy Dose Escalation in Unresectable Ewing Sarcoma: Final Results of a Phase 3 Randomized Controlled Trial.
Laskar S, Sinha S, Chatterjee A, Khanna N, Manjali JJ, Puri A, Gulia A, Nayak P, Vora T, Chinnaswamy G, Prasad M, Bajpai J, Juvekar S, Desai S, Janu A, Rangarajan V, Purandare N, Shah S, Rekhi B, Jambhekar N, Muckaden MA, Kurkure P
Int J Radiat Oncol Biol Phys 2022 Aug 1;113(5):996-1002. Epub 2022 May 11 doi: 10.1016/j.ijrobp.2022.04.024. PMID: 35568246
"Embryonic-type Neuroectodermal Tumor" Should Replace "Primitive Neuroectodermal Tumor" of the Testis and Gynecologic Tract: A Rationale for New Nomenclature.
Flood TA, Ulbright TM, Hirsch MS
Am J Surg Pathol 2021 Oct 1;45(10):1299-1302. doi: 10.1097/PAS.0000000000001703. PMID: 34232605
Temozolomide with irinotecan versus temozolomide, irinotecan plus bevacizumab for recurrent medulloblastoma of childhood: Report of a COG randomized Phase II screening trial.
Levy AS, Krailo M, Chi S, Villaluna D, Springer L, Williams-Hughes C, Fouladi M, Gajjar A
Pediatr Blood Cancer 2021 Aug;68(8):e29031. Epub 2021 Apr 12 doi: 10.1002/pbc.29031. PMID: 33844469Free PMC Article
Ewing Sarcoma and the History of Similar and Possibly Related Small Round Cell Tumors: From Whence Have We Come and Where are We Going?
Kilpatrick SE, Reith JD, Rubin B
Adv Anat Pathol 2018 Sep;25(5):314-326. doi: 10.1097/PAP.0000000000000203. PMID: 29911999
Sinonasal small round blue cell tumors: An approach to diagnosis.
Simons SA, Bridge JA, Leon ME
Semin Diagn Pathol 2016 Mar;33(2):91-103. Epub 2015 Sep 10 doi: 10.1053/j.semdp.2015.09.010. PMID: 26585346

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
Primary primitive neuroectodermal tumour of the kidney in adults: Experience of managing 12 cases with systematic review and pooled analysis of literature.
Sharma G, Kakkar N, Singh SK, Parmar K, Sharma AP, Mavuduru R
Int J Clin Pract 2021 Dec;75(12):e14971. Epub 2021 Oct 20 doi: 10.1111/ijcp.14971. PMID: 34626514
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
Proton Therapy in Children: A Systematic Review of Clinical Effectiveness in 15 Pediatric Cancers.
Leroy R, Benahmed N, Hulstaert F, Van Damme N, De Ruysscher D
Int J Radiat Oncol Biol Phys 2016 May 1;95(1):267-278. Epub 2015 Oct 17 doi: 10.1016/j.ijrobp.2015.10.025. PMID: 27084646
Embryonal tumor with abundant neuropil and true rosettes: a systematic literature review and report of 2 new cases.
Alexiou GA, Stefanaki K, Vartholomatos G, Sfakianos G, Prodromou N, Moschovi M
J Child Neurol 2013 Dec;28(12):1709-15. Epub 2013 Jan 17 doi: 10.1177/0883073812471434. PMID: 23334078

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