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Central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor

MedGen UID:
1389430
•Concept ID:
C4329632
•
Neoplastic Process
Synonyms: Central nervous system embryonal tumor; Central Nervous System Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor; Central nervous system primitive neuroectodermal tumor; central nervous system primitive neuroectodermal tumor; central nervous system primitive neuroectodermal tumour; CNS PNET
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)
 
Monarch Initiative: MONDO:0016713
Orphanet: ORPHA251870

Definition

A rare Ewing sarcoma/peripheral primitive neuroectodermal tumor that affects the central nervous system either as a primary dural neoplasm or by direct extension from adjacent soft tissues or bone. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCentral nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor

Professional guidelines

PubMed

The Children's Oncology Group Radiation Oncology Discipline: 15 Years of Contributions to the Treatment of Childhood Cancer.
Breneman JC, Donaldson SS, Constine L, Merchant T, Marcus K, Paulino AC, Followill D, Mahajan A, Laack N, Esiashvili N, Haas-Kogan D, Laurie F, Olch A, Ulin K, Hodgson D, Yock TI, Terezakis S, Krasin M, Panoff J, Chuba P, Hua CH, Hess CB, Houghton PJ, Wolden S, Buchsbaum J, Fitzgerald TJ, Kalapurakal JA
Int J Radiat Oncol Biol Phys 2018 Jul 15;101(4):860-874. Epub 2018 Mar 14 doi: 10.1016/j.ijrobp.2018.03.002. PMID: 29976498Free PMC Article
Management of pediatric cancer.
Pizzo PA
Hosp Pract (Off Ed) 1986 Mar 15;21(3):111-8, 124-30. doi: 10.1080/21548331.1986.11704942. PMID: 3005350
Central nervous system leukemia and solid tumors of childhood. Treatment with 1,3-bis (2-chloroethyl)-1-nitrosourea (BCNU).
Iriarte PV, Hananian J, Cortner JA
Cancer 1966 Sep;19(9):1187-94. doi: 10.1002/1097-0142(196609)19:9<1187::aid-cncr2820190902>3.0.co;2-t. PMID: 4288265

Supplemental Content

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