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Peripheral primitive neuroectodermal tumor

MedGen UID:
151926
Concept ID:
C0684337
Neoplastic Process
Synonyms: Peripheral neuroectodermal tumor; Peripheral primitive neuroectodermal neoplasm; Primitive neuroectodermal tumor (PNET) (type)
SNOMED CT: Peripheral neuroectodermal tumor (73676002); Peripheral primitive neuroectodermal tumor (73676002); Ewing sarcoma / peripheral neuroectodermal tumor (703707001); Ewing sarcoma / PNET (703707001); Peripheral neuroectodermal tumor (253096008); PPNET - peripheral primitive neuroectodermal tumor (73676002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
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

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

Professional guidelines

PubMed

Liu Y, Yuan Y, Zhang F, Hu K, Qiu J, Hou X, Yan J, Lian X, Sun S, Liu Z, Shen J
Sci Rep 2020 Sep 24;10(1):15656. doi: 10.1038/s41598-020-72680-6. PMID: 32973274Free PMC Article
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
Gururangan S, Marina NM, Luo X, Parham DM, Tzen CY, Greenwald CA, Rao BN, Kun LE, Meyer WH
J Pediatr Hematol Oncol 1998 Jan-Feb;20(1):55-61. doi: 10.1097/00043426-199801000-00009. PMID: 9482414

Recent clinical studies

Etiology

Peng J, Han XM
Medicine (Baltimore) 2022 Jul 22;101(29):e29564. doi: 10.1097/MD.0000000000029564. PMID: 35866758Free PMC Article
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
Li Q, Liu Y, Yu Y
Medicine (Baltimore) 2017 Dec;96(51):e9272. doi: 10.1097/MD.0000000000009272. PMID: 29390485Free PMC Article
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
Batsakis JG, Mackay B, el-Naggar AK
Ann Otol Rhinol Laryngol 1996 Oct;105(10):838-43. doi: 10.1177/000348949610501014. PMID: 8865780

Diagnosis

Keka-Sylaj A, Ramosaj A, Baloku A, Zogaj L, Mushica F, Kurshumliu F
J Med Case Rep 2022 Mar 31;16(1):128. doi: 10.1186/s13256-022-03354-2. PMID: 35354472Free PMC Article
Wang J, Li J, Zhang X, Zhang X, Xiao Y
BMC Cardiovasc Disord 2021 Jun 16;21(1):305. doi: 10.1186/s12872-021-02113-3. PMID: 34134636Free PMC Article
Li Y, Chen L, Zhou X, Gao L, Cai X, Yang C, Hu L
Eur J Ophthalmol 2021 Sep;31(5):NP65-NP73. Epub 2020 Jun 3 doi: 10.1177/1120672120926937. PMID: 32493126
Revannagowda S, Gangadhar K, Akaike G, Dighe M
Curr Probl Diagn Radiol 2020 Mar-Apr;49(2):133-139. Epub 2019 Jan 2 doi: 10.1067/j.cpradiol.2018.12.009. PMID: 30642618
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

Li Q, Liu Y, Yu Y
Medicine (Baltimore) 2017 Dec;96(51):e9272. doi: 10.1097/MD.0000000000009272. PMID: 29390485Free PMC Article
Heikaus S, Schaefer KL, Eucker J, Hogrebe E, Danebrock R, Wai DH, Krenn V, Gabbert HE, Poremba C
Hum Pathol 2009 Jun;40(6):893-7. Epub 2009 Mar 9 doi: 10.1016/j.humpath.2008.11.009. PMID: 19269015
Scurr M, Judson I
Oncologist 2006 Jan;11(1):65-72. doi: 10.1634/theoncologist.11-1-65. PMID: 16401715
Carvajal R, Meyers P
Hematol Oncol Clin North Am 2005 Jun;19(3):501-25, vi-vii. doi: 10.1016/j.hoc.2005.03.004. PMID: 15939194
Kovar H, Aryee D, Zoubek A
Curr Opin Oncol 1999 Jul;11(4):275-84. doi: 10.1097/00001622-199907000-00007. PMID: 10416880

Prognosis

Wang J, Li J, Zhang X, Zhang X, Xiao Y
BMC Cardiovasc Disord 2021 Jun 16;21(1):305. doi: 10.1186/s12872-021-02113-3. PMID: 34134636Free PMC Article
Deshpande G, Epari S, Gupta C, Shetty O, Gurav M, Chinnaswamy G, Moiyadi A, Gupta T
Childs Nerv Syst 2021 Mar;37(3):839-849. Epub 2020 Aug 6 doi: 10.1007/s00381-020-04850-w. PMID: 32761378
Liu Y, Yuan Y, Zhang F, Hu K, Qiu J, Hou X, Yan J, Lian X, Sun S, Liu Z, Shen J
Sci Rep 2020 Sep 24;10(1):15656. doi: 10.1038/s41598-020-72680-6. PMID: 32973274Free PMC Article
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
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

Clinical prediction guides

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
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
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
Pytel P, Lukas RV
Arch Pathol Lab Med 2009 Jul;133(7):1062-77. doi: 10.5858/133.7.1062. PMID: 19642733
Carvajal R, Meyers P
Hematol Oncol Clin North Am 2005 Jun;19(3):501-25, vi-vii. doi: 10.1016/j.hoc.2005.03.004. PMID: 15939194

Recent systematic reviews

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

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