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
Fetal brain tumors: a review of 154 cases.
Isaacs H
Am J Perinatol 2009 Jun;26(6):453-66. Epub 2009 Apr 24 doi: 10.1055/s-0029-1214245. PMID: 19396744
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
Immunohistochemistry of small round-cell tumors.
Devoe K, Weidner N
Semin Diagn Pathol 2000 Aug;17(3):216-24. PMID: 10968707
Chemotherapy for pediatric brain tumors.
Kedar A
Semin Pediatr Neurol 1997 Dec;4(4):320-32. doi: 10.1016/s1071-9091(97)80017-4. PMID: 9447624

Diagnosis

Cyclin D1 and Ewing's sarcoma/PNET: A microarray analysis.
Fagone P, Nicoletti F, Salvatorelli L, Musumeci G, Magro G
Acta Histochem 2015 Oct;117(8):824-8. Epub 2015 Sep 9 doi: 10.1016/j.acthis.2015.08.006. PMID: 26363896
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
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
Atypical teratoid/rhabdoid tumors.
Dang T, Vassilyadi M, Michaud J, Jimenez C, Ventureyra EC
Childs Nerv Syst 2003 Apr;19(4):244-8. Epub 2003 Apr 2 doi: 10.1007/s00381-003-0731-3. PMID: 12682757
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

Therapy

Refractory LCH With Secondary Intracranial PNET: A Case Report and Review of Literature.
Chayapathi V, Mahajan A, Kalra M
J Pediatr Hematol Oncol 2018 Mar;40(2):e117-e120. doi: 10.1097/MPH.0000000000000927. PMID: 28859038
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
Disseminated encephalomyelitis-like central nervous system neoplasm in childhood.
Zhao J, Bao X, Fu N, Ye J, Li T, Yuan Y, Zhang C, Zhang Y, Zhang Y, Qin J, Wu X
J Child Neurol 2014 Aug;29(8):NP28-34. Epub 2013 Jul 19 doi: 10.1177/0883073813495444. PMID: 23872916
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
Human neuroblastoma cells and 13-cis-retinoic acid.
Helson L, Helson C
J Neurooncol 1985;3(1):39-41. doi: 10.1007/BF00165170. PMID: 2987426

Prognosis

Cyclin D1 and Ewing's sarcoma/PNET: A microarray analysis.
Fagone P, Nicoletti F, Salvatorelli L, Musumeci G, Magro G
Acta Histochem 2015 Oct;117(8):824-8. Epub 2015 Sep 9 doi: 10.1016/j.acthis.2015.08.006. PMID: 26363896
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
Fetal brain tumors: a review of 154 cases.
Isaacs H
Am J Perinatol 2009 Jun;26(6):453-66. Epub 2009 Apr 24 doi: 10.1055/s-0029-1214245. PMID: 19396744
Atypical teratoid/rhabdoid tumors.
Dang T, Vassilyadi M, Michaud J, Jimenez C, Ventureyra EC
Childs Nerv Syst 2003 Apr;19(4):244-8. Epub 2003 Apr 2 doi: 10.1007/s00381-003-0731-3. PMID: 12682757
Cardiorespiratory decompensation following methylprednisolone administration.
Lucas KG, Howrie DL, Phebus CK
Pediatr Hematol Oncol 1993 Jul-Sep;10(3):249-55. doi: 10.3109/08880019309029492. PMID: 8217541

Clinical prediction guides

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
Cerebrospinal fluid disturbances after 381 consecutive craniotomies for intracranial tumors in pediatric patients.
Hosainey SA, Lassen B, Helseth E, Meling TR
J Neurosurg Pediatr 2014 Dec;14(6):604-14. Epub 2014 Oct 17 doi: 10.3171/2014.8.PEDS13585. PMID: 25325416
Medulloblastoma incidence has not changed over time: a CBTRUS study.
Partap S, Curran EK, Propp JM, Le GM, Sainani KL, Fisher PG
J Pediatr Hematol Oncol 2009 Dec;31(12):970-1. doi: 10.1097/MPH.0b013e3181bbc502. PMID: 19887963
Role of spinal MRI in the follow-up of children treated for medulloblastoma.
Bartels U, Shroff M, Sung L, Dag-Ellams U, Laperriere N, Rutka J, Bouffet E
Cancer 2006 Sep 15;107(6):1340-7. doi: 10.1002/cncr.22129. PMID: 16894528
Human neuroblastoma cells and 13-cis-retinoic acid.
Helson L, Helson C
J Neurooncol 1985;3(1):39-41. doi: 10.1007/BF00165170. PMID: 2987426

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...