U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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
Disrupting the CD47-SIRPα anti-phagocytic axis by a humanized anti-CD47 antibody is an efficacious treatment for malignant pediatric brain tumors.
Gholamin S, Mitra SS, Feroze AH, Liu J, Kahn SA, Zhang M, Esparza R, Richard C, Ramaswamy V, Remke M, Volkmer AK, Willingham S, Ponnuswami A, McCarty A, Lovelace P, Storm TA, Schubert S, Hutter G, Narayanan C, Chu P, Raabe EH, Harsh G 4th, Taylor MD, Monje M, Cho YJ, Majeti R, Volkmer JP, Fisher PG, Grant G, Steinberg GK, Vogel H, Edwards M, Weissman IL, Cheshier SH
Sci Transl Med 2017 Mar 15;9(381) doi: 10.1126/scitranslmed.aaf2968. PMID: 28298418

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
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
Pediatric vulvar malignancies: rare but important to know.
Strickland AL, Fadare O
Semin Diagn Pathol 2021 Jan;38(1):99-109. Epub 2020 Sep 5 doi: 10.1053/j.semdp.2020.09.001. PMID: 32943238
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
Primary thoracic sarcomas.
Gladish GW, Sabloff BM, Munden RF, Truong MT, Erasmus JJ, Chasen MH
Radiographics 2002 May-Jun;22(3):621-37. doi: 10.1148/radiographics.22.3.g02ma17621. PMID: 12006691

Diagnosis

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
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

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
Disrupting the CD47-SIRPα anti-phagocytic axis by a humanized anti-CD47 antibody is an efficacious treatment for malignant pediatric brain tumors.
Gholamin S, Mitra SS, Feroze AH, Liu J, Kahn SA, Zhang M, Esparza R, Richard C, Ramaswamy V, Remke M, Volkmer AK, Willingham S, Ponnuswami A, McCarty A, Lovelace P, Storm TA, Schubert S, Hutter G, Narayanan C, Chu P, Raabe EH, Harsh G 4th, Taylor MD, Monje M, Cho YJ, Majeti R, Volkmer JP, Fisher PG, Grant G, Steinberg GK, Vogel H, Edwards M, Weissman IL, Cheshier SH
Sci Transl Med 2017 Mar 15;9(381) doi: 10.1126/scitranslmed.aaf2968. PMID: 28298418
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
Pediatric cerebral ganglioneuroblastoma.
Steenberge SP, Prayson RA
J Clin Neurosci 2014 Nov;21(11):2023-5. Epub 2014 Sep 10 doi: 10.1016/j.jocn.2014.07.007. PMID: 25216629
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

The role of SPARC/ON in human osteosarcoma.
Sergi CM
Adv Protein Chem Struct Biol 2023;133:181-192. Epub 2022 Nov 25 doi: 10.1016/bs.apcsb.2022.10.007. PMID: 36707201
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
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

"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
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
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
Epidemiology and etiology of gliomas.
Ohgaki H, Kleihues P
Acta Neuropathol 2005 Jan;109(1):93-108. Epub 2005 Feb 1 doi: 10.1007/s00401-005-0991-y. PMID: 15685439

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

Supplemental Content

Table of contents

    Clinical resources

    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.

    Consumer resources

    Reviews

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...