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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
Treatment of children with peripheral primitive neuroectodermal tumor or extraosseous Ewing's tumor with Ewing's-directed therapy.
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

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
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
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
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 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

Peripheral primitive neuroectodermal tumor: a case report.
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
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
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 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
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
The Ewing family of tumors and the search for the Achilles' heel.
Kovar H, Aryee D, Zoubek A
Curr Opin Oncol 1999 Jul;11(4):275-84. doi: 10.1097/00001622-199907000-00007. PMID: 10416880
Treatment of children with peripheral primitive neuroectodermal tumor or extraosseous Ewing's tumor with Ewing's-directed therapy.
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

Prognosis

Primitive neuroectodermal tumor of the pericardium: a case report and literature review.
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
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
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
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
Update on diagnostic practice: tumors of the nervous system.
Pytel P, Lukas RV
Arch Pathol Lab Med 2009 Jul;133(7):1062-77. doi: 10.5858/133.7.1062. PMID: 19642733
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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