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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.
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

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

Professional guidelines

PubMed

Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor.
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
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
Peripheral primitive neuroectodermal tumor: challenge for multimodal treatment.
Zimmermann T, Blütters-Sawatzki R, Flechsenhar K, Padberg WM
World J Surg 2001 Nov;25(11):1367-72. doi: 10.1007/s00268-001-0142-0. PMID: 11760735

Recent clinical studies

Etiology

Spinal Peripheral Primitive Neuroectodermal Tumors: A Radiological Analysis of Ten Cases.
Yi X, Zhou M, Liao S, Gong G, Yin H, Chen Y, Liao W
Turk Neurosurg 2019;29(1):33-42. doi: 10.5137/1019-5149.JTN.21931-17.2. PMID: 29492943
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
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
Peripheral primitive neuroectodermal tumor/Ewing's sarcoma of the craniospinal vault: case reports and review.
Mobley BC, Roulston D, Shah GV, Bijwaard KE, McKeever PE
Hum Pathol 2006 Jul;37(7):845-53. Epub 2006 May 19 doi: 10.1016/j.humpath.2006.02.011. PMID: 16784984
Ewing's sarcoma and peripheral primitive neuroectodermal tumor: an interim report.
Batsakis JG, Mackay B, el-Naggar AK
Ann Otol Rhinol Laryngol 1996 Oct;105(10):838-43. doi: 10.1177/000348949610501014. PMID: 8865780

Diagnosis

Primary Intra-abdominal Ewing's Sarcoma in Adults: A Multimodality Imaging Spectrum.
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
Sinonasal small round blue cell tumors: An approach to diagnosis.
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
Congenital Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature.
Jin SG, Jiang XP, Zhong L
Pediatr Neonatol 2016 Oct;57(5):436-439. Epub 2014 Jan 27 doi: 10.1016/j.pedneo.2013.11.002. PMID: 24480101
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
Cytogenetic and experimental models.
Fletcher J
Curr Opin Oncol 1992 Aug;4(4):659-66. doi: 10.1097/00001622-199208000-00010. PMID: 1511022

Therapy

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
Primary peripheral primitive neuroectodermal tumor/Ewing's tumor of the testis in a 46-year-old man-differential diagnosis and review of the literature.
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
How to treat the Ewing's family of sarcomas in adult patients.
Scurr M, Judson I
Oncologist 2006 Jan;11(1):65-72. doi: 10.1634/theoncologist.11-1-65. PMID: 16401715
Ewing's sarcoma and primitive neuroectodermal family of tumors.
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
Ewing's sarcoma and peripheral primitive neuroectodermal tumor.
Jürgens HF
Curr Opin Oncol 1994 Jul;6(4):391-6. PMID: 7803540

Prognosis

Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor.
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
Sinonasal small round blue cell tumors: An approach to diagnosis.
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
Congenital Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature.
Jin SG, Jiang XP, Zhong L
Pediatr Neonatol 2016 Oct;57(5):436-439. Epub 2014 Jan 27 doi: 10.1016/j.pedneo.2013.11.002. PMID: 24480101
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
Cytogenetic and experimental models.
Fletcher J
Curr Opin Oncol 1992 Aug;4(4):659-66. doi: 10.1097/00001622-199208000-00010. PMID: 1511022

Clinical prediction guides

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
Computed Tomography and Magnetic Resonance Imaging Characteristics of Peripheral Primitive Neuroectodermal Tumor: A Retrospective Analysis of 16 Cases.
Hou W, Xu L, Zhan H, Wang H, Xu M, Yu Y
J Comput Assist Tomogr 2017 Mar/Apr;41(2):224-230. doi: 10.1097/RCT.0000000000000496. PMID: 27680421
Sinonasal small round blue cell tumors: An approach to diagnosis.
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
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
Ewing's sarcoma and primitive neuroectodermal family of tumors.
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

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

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