U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Peripheral primitive neuroectodermal tumor of soft tissues

MedGen UID:
205115
•Concept ID:
C1112437
•
Neoplastic Process
Synonyms: Peripheral Neuroectodermal Tumor of Soft Tissues; peripheral neuroectodermal tumor of soft tissues; Peripheral Neuroectodermal Tumor of the Soft Tissues; peripheral neuroectodermal tumor of the soft tissues; peripheral neuroectodermal tumour of soft tissues; peripheral neuroectodermal tumour of the soft tissues; Peripheral Neuroepithelioma of Soft Tissues; peripheral neuroepithelioma of soft tissues; Peripheral Neuroepithelioma of the Soft Tissues; peripheral neuroepithelioma of the soft tissues; Peripheral Primitive Neuroectodermal Tumor of Soft Tissues; peripheral primitive neuroectodermal tumor of soft tissues; soft tissue peripheral neuroepithelioma
 
Monarch Initiative: MONDO:0002982

Definition

A small round cell tumor with neural differentiation arising from the soft tissues. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPeripheral primitive neuroectodermal tumor of soft tissues

Professional guidelines

PubMed

The new international neuroblastoma response criteria.
Orr KE, McHugh K
Pediatr Radiol 2019 Oct;49(11):1433-1440. Epub 2019 Oct 16 doi: 10.1007/s00247-019-04397-2. PMID: 31620844
Treatment and outcome of adult-onset neuroblastoma.
Suzuki M, Kushner BH, Kramer K, Basu EM, Roberts SS, Hammond WJ, LaQuaglia MP, Wolden SL, Cheung NV, Modak S
Int J Cancer 2018 Sep 1;143(5):1249-1258. Epub 2018 Apr 6 doi: 10.1002/ijc.31399. PMID: 29574715Free PMC Article
Revisions to the International Neuroblastoma Response Criteria: A Consensus Statement From the National Cancer Institute Clinical Trials Planning Meeting.
Park JR, Bagatell R, Cohn SL, Pearson AD, Villablanca JG, Berthold F, Burchill S, Boubaker A, McHugh K, Nuchtern JG, London WB, Seibel NL, Lindwasser OW, Maris JM, Brock P, Schleiermacher G, Ladenstein R, Matthay KK, Valteau-Couanet D
J Clin Oncol 2017 Aug 1;35(22):2580-2587. Epub 2017 May 4 doi: 10.1200/JCO.2016.72.0177. PMID: 28471719Free PMC Article

Recent clinical studies

Etiology

Treatment and outcome of adult-onset neuroblastoma.
Suzuki M, Kushner BH, Kramer K, Basu EM, Roberts SS, Hammond WJ, LaQuaglia MP, Wolden SL, Cheung NV, Modak S
Int J Cancer 2018 Sep 1;143(5):1249-1258. Epub 2018 Apr 6 doi: 10.1002/ijc.31399. PMID: 29574715Free PMC Article
Revisions to the International Neuroblastoma Response Criteria: A Consensus Statement From the National Cancer Institute Clinical Trials Planning Meeting.
Park JR, Bagatell R, Cohn SL, Pearson AD, Villablanca JG, Berthold F, Burchill S, Boubaker A, McHugh K, Nuchtern JG, London WB, Seibel NL, Lindwasser OW, Maris JM, Brock P, Schleiermacher G, Ladenstein R, Matthay KK, Valteau-Couanet D
J Clin Oncol 2017 Aug 1;35(22):2580-2587. Epub 2017 May 4 doi: 10.1200/JCO.2016.72.0177. PMID: 28471719Free PMC Article
Phenotype and immunophenotype of the most common pediatric tumors.
Picarsic J, Reyes-Múgica M
Appl Immunohistochem Mol Morphol 2015 May-Jun;23(5):313-26. doi: 10.1097/PAI.0000000000000068. PMID: 25390357
Neonatal cancer.
Orbach D, Sarnacki S, Brisse HJ, Gauthier-Villars M, Jarreau PH, Tsatsaris V, Baruchel A, Zerah M, Seigneur E, Peuchmaur M, Doz F
Lancet Oncol 2013 Dec;14(13):e609-20. doi: 10.1016/S1470-2045(13)70236-5. PMID: 24275134
Pediatrics: diagnosis of neuroblastoma.
Sharp SE, Gelfand MJ, Shulkin BL
Semin Nucl Med 2011 Sep;41(5):345-53. doi: 10.1053/j.semnuclmed.2011.05.001. PMID: 21803184

Diagnosis

The new international neuroblastoma response criteria.
Orr KE, McHugh K
Pediatr Radiol 2019 Oct;49(11):1433-1440. Epub 2019 Oct 16 doi: 10.1007/s00247-019-04397-2. PMID: 31620844
Revisions to the International Neuroblastoma Response Criteria: A Consensus Statement From the National Cancer Institute Clinical Trials Planning Meeting.
Park JR, Bagatell R, Cohn SL, Pearson AD, Villablanca JG, Berthold F, Burchill S, Boubaker A, McHugh K, Nuchtern JG, London WB, Seibel NL, Lindwasser OW, Maris JM, Brock P, Schleiermacher G, Ladenstein R, Matthay KK, Valteau-Couanet D
J Clin Oncol 2017 Aug 1;35(22):2580-2587. Epub 2017 May 4 doi: 10.1200/JCO.2016.72.0177. PMID: 28471719Free PMC Article
Diagnostic Imaging of Fetal and Neonatal Abdominal and Soft Tissue Tumors.
Nagaraj UD, Kline-Fath BM
Curr Pediatr Rev 2015;11(3):143-50. doi: 10.2174/1573396311666150714110605. PMID: 26168940
Neonatal cancer.
Orbach D, Sarnacki S, Brisse HJ, Gauthier-Villars M, Jarreau PH, Tsatsaris V, Baruchel A, Zerah M, Seigneur E, Peuchmaur M, Doz F
Lancet Oncol 2013 Dec;14(13):e609-20. doi: 10.1016/S1470-2045(13)70236-5. PMID: 24275134
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

Therapy

Treatment and outcome of adult-onset neuroblastoma.
Suzuki M, Kushner BH, Kramer K, Basu EM, Roberts SS, Hammond WJ, LaQuaglia MP, Wolden SL, Cheung NV, Modak S
Int J Cancer 2018 Sep 1;143(5):1249-1258. Epub 2018 Apr 6 doi: 10.1002/ijc.31399. PMID: 29574715Free PMC Article
Antiangiogenic therapy for primitive neuroectodermal tumor with thalidomide: A case report and review of literature.
Li Q, Liu Y, Yu Y
Medicine (Baltimore) 2017 Dec;96(51):e9272. doi: 10.1097/MD.0000000000009272. PMID: 29390485Free PMC Article
Revisions to the International Neuroblastoma Response Criteria: A Consensus Statement From the National Cancer Institute Clinical Trials Planning Meeting.
Park JR, Bagatell R, Cohn SL, Pearson AD, Villablanca JG, Berthold F, Burchill S, Boubaker A, McHugh K, Nuchtern JG, London WB, Seibel NL, Lindwasser OW, Maris JM, Brock P, Schleiermacher G, Ladenstein R, Matthay KK, Valteau-Couanet D
J Clin Oncol 2017 Aug 1;35(22):2580-2587. Epub 2017 May 4 doi: 10.1200/JCO.2016.72.0177. PMID: 28471719Free PMC Article
¹³¹I-Metaiodobenzylguanidine Theranostics in Neuroblastoma: Historical Perspectives; Practical Applications.
Parisi MT, Eslamy H, Park JR, Shulkin BL, Yanik GA
Semin Nucl Med 2016 May;46(3):184-202. doi: 10.1053/j.semnuclmed.2016.02.002. PMID: 27067500
Pesticides and childhood cancer.
Zahm SH, Ward MH
Environ Health Perspect 1998 Jun;106 Suppl 3(Suppl 3):893-908. doi: 10.1289/ehp.98106893. PMID: 9646054Free PMC Article

Prognosis

Treatment and outcome of adult-onset neuroblastoma.
Suzuki M, Kushner BH, Kramer K, Basu EM, Roberts SS, Hammond WJ, LaQuaglia MP, Wolden SL, Cheung NV, Modak S
Int J Cancer 2018 Sep 1;143(5):1249-1258. Epub 2018 Apr 6 doi: 10.1002/ijc.31399. PMID: 29574715Free PMC Article
Diagnostic Imaging of Fetal and Neonatal Abdominal and Soft Tissue Tumors.
Nagaraj UD, Kline-Fath BM
Curr Pediatr Rev 2015;11(3):143-50. doi: 10.2174/1573396311666150714110605. PMID: 26168940
Phenotype and immunophenotype of the most common pediatric tumors.
Picarsic J, Reyes-Múgica M
Appl Immunohistochem Mol Morphol 2015 May-Jun;23(5):313-26. doi: 10.1097/PAI.0000000000000068. PMID: 25390357
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
Pediatrics: diagnosis of neuroblastoma.
Sharp SE, Gelfand MJ, Shulkin BL
Semin Nucl Med 2011 Sep;41(5):345-53. doi: 10.1053/j.semnuclmed.2011.05.001. PMID: 21803184

Clinical prediction guides

Postchemotherapy gross residual tumor in non-high-risk neuroblastoma: Clinical significance and the role of adjuvant therapy.
Seo ES, Lim H, Cho HW, Ju HY, Lee JW, Yoo KH, Lee S, Lim DH, Sung KW, Koo HH
Pediatr Blood Cancer 2022 Nov;69(11):e29840. Epub 2022 Jun 23 doi: 10.1002/pbc.29840. PMID: 35735763
¹³¹I-Metaiodobenzylguanidine Theranostics in Neuroblastoma: Historical Perspectives; Practical Applications.
Parisi MT, Eslamy H, Park JR, Shulkin BL, Yanik GA
Semin Nucl Med 2016 May;46(3):184-202. doi: 10.1053/j.semnuclmed.2016.02.002. PMID: 27067500
Radiologic and clinicopathologic findings of peripheral primitive neuroectodermal tumors.
Ba L, Tan H, Xiao H, Guan Y, Gao J, Gao X
Acta Radiol 2015 Jul;56(7):820-8. Epub 2014 Jul 29 doi: 10.1177/0284185114539321. PMID: 25073463
Ifosfamide in pediatric solid tumors.
Carli M, Passone E, Perilongo G, Bisogno G
Oncology 2003;65 Suppl 2:99-104. doi: 10.1159/000073369. PMID: 14586158
Pesticides and childhood cancer.
Zahm SH, Ward MH
Environ Health Perspect 1998 Jun;106 Suppl 3(Suppl 3):893-908. doi: 10.1289/ehp.98106893. PMID: 9646054Free PMC Article

Recent systematic reviews

Malignant spinal cord compression in the paediatric population-a systematic review, meta-analysis.
Quraishi NA, Palliyil N, Hassanin MA, D'Aquino D, Shetaiwi A, Walker D
Eur Spine J 2023 Dec;32(12):4306-4313. Epub 2023 Jun 20 doi: 10.1007/s00586-023-07820-3. PMID: 37338630

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

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...