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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

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
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
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

Kushner BH, LaQuaglia MP, Cardenas FI, Basu EM, Gerstle JT, Kramer K, Roberts SS, Wolden SL, Cheung NV, Modak S
Int J Cancer 2023 Dec 15;153(12):2019-2031. Epub 2023 Aug 21 doi: 10.1002/ijc.34693. PMID: 37602920
Zhao Q, Liu Y, Zhang Y, Meng L, Wei J, Wang B, Wang H, Xin Y, Dong L, Jiang X
Crit Rev Oncol Hematol 2020 May;149:102924. Epub 2020 Mar 3 doi: 10.1016/j.critrevonc.2020.102924. PMID: 32172225
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
Picarsic J, Reyes-Múgica M
Appl Immunohistochem Mol Morphol 2015 May-Jun;23(5):313-26. doi: 10.1097/PAI.0000000000000068. PMID: 25390357
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

Diagnosis

Kushner BH, LaQuaglia MP, Cardenas FI, Basu EM, Gerstle JT, Kramer K, Roberts SS, Wolden SL, Cheung NV, Modak S
Int J Cancer 2023 Dec 15;153(12):2019-2031. Epub 2023 Aug 21 doi: 10.1002/ijc.34693. PMID: 37602920
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
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
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
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

Samim A, Blom T, Poot AJ, Windhorst AD, Fiocco M, Tolboom N, Braat AJAT, Viol SLM, van Rooij R, van Noesel MM, Lam MGEH, Tytgat GAM, de Keizer B
Eur J Nucl Med Mol Imaging 2023 Mar;50(4):1146-1157. Epub 2022 Dec 12 doi: 10.1007/s00259-022-06063-6. PMID: 36504277Free PMC Article
Li Q, Liu Y, Yu Y
Medicine (Baltimore) 2017 Dec;96(51):e9272. doi: 10.1097/MD.0000000000009272. PMID: 29390485Free PMC Article
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
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
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

Kushner BH, LaQuaglia MP, Cardenas FI, Basu EM, Gerstle JT, Kramer K, Roberts SS, Wolden SL, Cheung NV, Modak S
Int J Cancer 2023 Dec 15;153(12):2019-2031. Epub 2023 Aug 21 doi: 10.1002/ijc.34693. PMID: 37602920
Nagaraj UD, Kline-Fath BM
Curr Pediatr Rev 2015;11(3):143-50. doi: 10.2174/1573396311666150714110605. PMID: 26168940
Picarsic J, Reyes-Múgica M
Appl Immunohistochem Mol Morphol 2015 May-Jun;23(5):313-26. doi: 10.1097/PAI.0000000000000068. PMID: 25390357
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
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

Zhou H, He S, Zhang D, Wang J, Yang X, Jiao J, Xu W, Yang J, Xiao J
Medicine (Baltimore) 2023 Oct 27;102(43):e34231. doi: 10.1097/MD.0000000000034231. PMID: 37904412Free PMC Article
Samim A, Blom T, Poot AJ, Windhorst AD, Fiocco M, Tolboom N, Braat AJAT, Viol SLM, van Rooij R, van Noesel MM, Lam MGEH, Tytgat GAM, de Keizer B
Eur J Nucl Med Mol Imaging 2023 Mar;50(4):1146-1157. Epub 2022 Dec 12 doi: 10.1007/s00259-022-06063-6. PMID: 36504277Free PMC Article
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
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
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

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

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