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Peripheral primitive neuroectodermal neoplasm

MedGen UID:: 151926
•Concept ID:: C0684337
•: Neoplastic Process

Synonyms:	Peripheral neuroectodermal tumor; Peripheral primitive neuroectodermal tumor; Primitive neuroectodermal tumor (PNET) (type)
SNOMED CT:	Peripheral neuroectodermal tumor (73676002); Peripheral primitive neuroectodermal tumor (73676002); PPNET (73676002); Ewing sarcoma / peripheral neuroectodermal tumor (703707001); Ewing sarcoma / PNET (703707001); Peripheral neuroectodermal tumor (253096008)
Modes of inheritance:	Not genetically inherited MedGen UID: 988794 •Concept ID: CN307044 • Finding Source: Orphanet clinical entity without genetic inheritance. See: This record Not genetically inherited (Orphanet)

HPO:	HP:0030067
Monarch Initiative:	MONDO:0018271
Orphanet:	ORPHA370348

Definition

A primitive neuroectodermal neoplasm that occurs extracranially in soft tissue and bone. [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVPeripheral primitive neuroectodermal neoplasm

Disorder by Site
- Connective and Soft Tissue Disorder
  - Connective tissue disorder
    - Bone Neoplasm
      - Malignant Bone Neoplasm
        Bone sarcoma
        Peripheral primitive neuroectodermal neoplasm
        Askin tumor
        Localized Askin Tumor
        Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
        Recurrent Ewing Sarcoma
        Recurrent Peripheral Primitive Neuroectodermal Tumor
        Localized Peripheral Primitive Neuroectodermal Tumor
        Localized Peripheral Primitive Neuroectodermal Tumor of Bone
        Neuroblastoma
        Cerebral neuroblastoma
        Childhood Central Nervous System Neuroblastoma
        Childhood Neuroblastoma
        Congenital neuroblastoma
        Differentiating neuroblastoma
        Extracranial neuroblastoma
        Olfactory esthesioneuroblastoma
        Peripheral Neuroblastoma
        Spinal cord neuroblastoma
        Familial Neuroblastoma
        Ganglioneuroblastoma
        Central Nervous System Ganglioneuroblastoma
        Childhood Ganglioneuroblastoma
        Intermixed schwannian stroma-rich ganglioneuroblastoma
        Nodular ganglioneuroblastoma
        Peripheral ganglioneuroblastoma
        Recurrent Ganglioneuroblastoma
        Refractory Ganglioneuroblastoma
        High Risk Neuroblastoma
        Localized neuroblastoma
        Metastatic Neuroblastoma
        Neuroblastoma, susceptibility to, 1
        Neuroblastoma, susceptibility to, 2
        Neuroblastoma, susceptibility to, 3
        Poorly Differentiated Neuroblastoma
        Recurrent Neuroblastoma
        Recurrent Olfactory Neuroblastoma
        Refractory Neuroblastoma
        Regional Neuroblastoma
        Resectable Neuroblastoma
        Localized Resectable Neuroblastoma
        Retinoblastoma
        bilateral retinoblastoma
        Differentiated Retinoblastoma
        Diffuse Retinoblastoma
        Extraocular retinoblastoma
        Hereditary retinoblastoma
        Intraocular retinoblastoma
        Non-hereditary retinoblastoma
        Recurrent Retinoblastoma
        Retinoblastoma by AJCC v8 Stage
        Sporadic Retinoblastoma
        Trilateral retinoblastoma
        Undifferentiated Retinoblastoma
        Unilateral retinoblastoma
        Sellar Neuroblastoma
        Stage 1 Neuroblastoma
        Stage 2 Neuroblastoma
        Stage 2A Neuroblastoma
        Stage 2B Neuroblastoma
        Stage 3 Neuroblastoma
        Stage 4 Neuroblastoma
        Stage 4S Neuroblastoma
        Undifferentiated Neuroblastoma
        Unresectable Neuroblastoma
        Localized Unresectable Neuroblastoma
        Peripheral primitive neuroectodermal tumor of bone
        Metastatic Peripheral Primitive Neuroectodermal Tumor of Bone
        Recurrent Peripheral Primitive Neuroectodermal Tumor of Bone
        Peripheral primitive neuroectodermal tumor of soft tissues
        Peripheral Primitive Neuroectodermal Tumor of the Kidney
        Refractory Peripheral Primitive Neuroectodermal Tumor

Professional guidelines

PubMed

Diagnosis and Management of Opsoclonus-Myoclonus-Ataxia Syndrome in Children: An International Perspective.

Rossor T, Yeh EA, Khakoo Y, Angelini P, Hemingway C, Irani SR, Schleiermacher G, Santosh P, Lotze T, Dale RC, Deiva K, Hero B, Klein A, de Alarcon P, Gorman MP, Mitchell WG, Lim M; OMS Study Group
Neurol Neuroimmunol Neuroinflamm 2022 May;9(3) Epub 2022 Mar 8 doi: 10.1212/NXI.0000000000001153. PMID: 35260471 Free PMC Article

Management of Neuroblastoma in Pediatric Patients.

Croteau N, Nuchtern J, LaQuaglia MP
Surg Oncol Clin N Am 2021 Apr;30(2):291-304. Epub 2021 Feb 10 doi: 10.1016/j.soc.2020.11.010. PMID: 33706901

Overview and recent advances in the treatment of neuroblastoma.

Whittle SB, Smith V, Doherty E, Zhao S, McCarty S, Zage PE
Expert Rev Anticancer Ther 2017 Apr;17(4):369-386. Epub 2017 Mar 15 doi: 10.1080/14737140.2017.1285230. PMID: 28142287

See all (909)

Recent clinical studies

Etiology

Revised Neuroblastoma Risk Classification System: A Report From the Children's Oncology Group.

Irwin MS, Naranjo A, Zhang FF, Cohn SL, London WB, Gastier-Foster JM, Ramirez NC, Pfau R, Reshmi S, Wagner E, Nuchtern J, Asgharzadeh S, Shimada H, Maris JM, Bagatell R, Park JR, Hogarty MD
J Clin Oncol 2021 Oct 10;39(29):3229-3241. Epub 2021 Jul 28 doi: 10.1200/JCO.21.00278. PMID: 34319759 Free PMC Article

Neuroblastoma.

Chung C, Boterberg T, Lucas J, Panoff J, Valteau-Couanet D, Hero B, Bagatell R, Hill-Kayser CE
Pediatr Blood Cancer 2021 May;68 Suppl 2(Suppl 2):e28473. doi: 10.1002/pbc.28473. PMID: 33818884 Free PMC Article

Neonatal Neuroblastoma.

Davidoff AM
Clin Perinatol 2021 Mar;48(1):101-115. Epub 2021 Jan 12 doi: 10.1016/j.clp.2020.11.006. PMID: 33583499

Neuroblastoma.

Nakagawara A, Li Y, Izumi H, Muramori K, Inada H, Nishi M
Jpn J Clin Oncol 2018 Mar 1;48(3):214-241. doi: 10.1093/jjco/hyx176. PMID: 29378002

Overview and recent advances in the treatment of neuroblastoma.

Whittle SB, Smith V, Doherty E, Zhao S, McCarty S, Zage PE
Expert Rev Anticancer Ther 2017 Apr;17(4):369-386. Epub 2017 Mar 15 doi: 10.1080/14737140.2017.1285230. PMID: 28142287

See all (5910)

Diagnosis

Imaging spectrum of peripheral primitive neuroectodermal tumours.

Gupta P, Hari S, Thulkar S
Singapore Med J 2013 Aug;54(8):463-2. doi: 10.11622/smedj.2013155. PMID: 24005454

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: 22420726 Free PMC Article

Esthesioneuroblastoma.

Shah JP, Feghali J
CA Cancer J Clin 1983 May-Jun;33(3):154-9. doi: 10.3322/canjclin.33.3.154. PMID: 6404523

Esthesioneuroblastoma.

Shah JP, Feghali J
Am J Surg 1981 Oct;142(4):456-8. doi: 10.1016/0002-9610(81)90374-3. PMID: 7283046

Esthesioneuroblastoma.

Elkon D, Hightower SI, Lim ML, Cantrell RW, Constable WC
Cancer 1979 Sep;44(3):1087-94. doi: 10.1002/1097-0142(197909)44:3<1087::aid-cncr2820440343>3.0.co;2-a. PMID: 383268

See all (7484)

Therapy

Revised Neuroblastoma Risk Classification System: A Report From the Children's Oncology Group.

Neuroblastoma.

Nakagawara A, Li Y, Izumi H, Muramori K, Inada H, Nishi M
Jpn J Clin Oncol 2018 Mar 1;48(3):214-241. doi: 10.1093/jjco/hyx176. PMID: 29378002

Advances in Risk Classification and Treatment Strategies for Neuroblastoma.

Pinto NR, Applebaum MA, Volchenboum SL, Matthay KK, London WB, Ambros PF, Nakagawara A, Berthold F, Schleiermacher G, Park JR, Valteau-Couanet D, Pearson AD, Cohn SL
J Clin Oncol 2015 Sep 20;33(27):3008-17. Epub 2015 Aug 24 doi: 10.1200/JCO.2014.59.4648. PMID: 26304901 Free PMC Article

Anti-GD2 antibody with GM-CSF, interleukin-2, and isotretinoin for neuroblastoma.

Yu AL, Gilman AL, Ozkaynak MF, London WB, Kreissman SG, Chen HX, Smith M, Anderson B, Villablanca JG, Matthay KK, Shimada H, Grupp SA, Seeger R, Reynolds CP, Buxton A, Reisfeld RA, Gillies SD, Cohn SL, Maris JM, Sondel PM; Children's Oncology Group
N Engl J Med 2010 Sep 30;363(14):1324-34. doi: 10.1056/NEJMoa0911123. PMID: 20879881 Free PMC Article

Recent advances in neuroblastoma.

Maris JM
N Engl J Med 2010 Jun 10;362(23):2202-11. doi: 10.1056/NEJMra0804577. PMID: 20558371 Free PMC Article

See all (4629)

Prognosis

Gangliosides and Neuroblastomas.

Schengrund CL
Int J Mol Sci 2020 Jul 27;21(15) doi: 10.3390/ijms21155313. PMID: 32726962 Free PMC Article

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: 22420726 Free PMC Article

Neuroblastoma.

Alexander F
Urol Clin North Am 2000 Aug;27(3):383-92, vii. doi: 10.1016/s0094-0143(05)70087-2. PMID: 10985139

Neuroblastoma.

Shah S, Ravindranath Y
Indian J Pediatr 1998 Sep-Oct;65(5):691-705. doi: 10.1007/BF02731044. PMID: 10773924

Esthesioneuroblastoma.

Elkon D, Hightower SI, Lim ML, Cantrell RW, Constable WC
Cancer 1979 Sep;44(3):1087-94. doi: 10.1002/1097-0142(197909)44:3<1087::aid-cncr2820440343>3.0.co;2-a. PMID: 383268

See all (5908)

Clinical prediction guides

Neonatal Neuroblastoma.

Davidoff AM
Clin Perinatol 2021 Mar;48(1):101-115. Epub 2021 Jan 12 doi: 10.1016/j.clp.2020.11.006. PMID: 33583499

Neuroblastoma Origin and Therapeutic Targets for Immunotherapy.

Kholodenko IV, Kalinovsky DV, Doronin II, Deyev SM, Kholodenko RV
J Immunol Res 2018;2018:7394268. Epub 2018 Jul 11 doi: 10.1155/2018/7394268. PMID: 30116755 Free PMC Article

Biological and Genetic Features of Neuroblastoma and Their Clinical Importance.

Aygun N
Curr Pediatr Rev 2018;14(2):73-90. doi: 10.2174/1573396314666180129101627. PMID: 29380702

Management of Neuroblastoma: ICMR Consensus Document.

Bansal D, Totadri S, Chinnaswamy G, Agarwala S, Vora T, Arora B, Prasad M, Kapoor G, Radhakrishnan V, Laskar S, Kaur T, Rath GK, Bakhshi S
Indian J Pediatr 2017 Jun;84(6):446-455. Epub 2017 Apr 3 doi: 10.1007/s12098-017-2298-0. PMID: 28367616

Advances in Risk Classification and Treatment Strategies for Neuroblastoma.

See all (5443)

Recent systematic reviews

The SH-SY5Y human neuroblastoma cell line, a relevant in vitro cell model for investigating neurotoxicology in human: Focus on organic pollutants.

Lopez-Suarez L, Awabdh SA, Coumoul X, Chauvet C
Neurotoxicology 2022 Sep;92:131-155. Epub 2022 Jul 29 doi: 10.1016/j.neuro.2022.07.008. PMID: 35914637

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

ROHHAD(NET) Syndrome: Systematic Review of the Clinical Timeline and Recommendations for Diagnosis and Prognosis.

Harvengt J, Gernay C, Mastouri M, Farhat N, Lebrethon MC, Seghaye MC, Bours V
J Clin Endocrinol Metab 2020 Jul 1;105(7) doi: 10.1210/clinem/dgaa247. PMID: 32407531

Tobacco, alcohol and illicit drugs during pregnancy and risk of neuroblastoma: systematic review.

Müller-Schulte E, Kurlemann G, Harder A
Arch Dis Child Fetal Neonatal Ed 2018 Sep;103(5):F467-F473. Epub 2017 Nov 21 doi: 10.1136/archdischild-2017-313615. PMID: 29162685

Thoracoscopic procedures in pediatric surgery: what is the evidence?

Dingemann C, Ure B, Dingemann J
Eur J Pediatr Surg 2014 Feb;24(1):14-9. Epub 2013 Jul 12 doi: 10.1055/s-0033-1350060. PMID: 23852720

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MedGen

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Peripheral primitive neuroectodermal neoplasm

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

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