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Central Nervous System Embryonal Tumor

MedGen UID:
234214
•Concept ID:
C1333378
•
Neoplastic Process
Synonyms: Central Nervous System Embryonal Neoplasm; CNS Embryonal Neoplasm; CNS Embryonal Tumor; Embryonal Neoplasm of Central Nervous System; Embryonal Neoplasm of CNS; Embryonal Neoplasm of the Central Nervous System; Embryonal Neoplasm of the CNS; Embryonal Tumor of Central Nervous System; Embryonal Tumor of CNS; Embryonal Tumor of the Central Nervous System; Embryonal Tumor of the CNS

Definition

A malignant neoplasm of embryonal origin, arising from the central nervous system. It usually affects children. Representative examples include ependymoblastoma, medulloblastoma, and atypical teratoid/rhabdoid tumor. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCentral Nervous System Embryonal Tumor

Professional guidelines

PubMed

EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood.
Weller M, van den Bent M, Preusser M, Le Rhun E, Tonn JC, Minniti G, Bendszus M, Balana C, Chinot O, Dirven L, French P, Hegi ME, Jakola AS, Platten M, Roth P, Rudà R, Short S, Smits M, Taphoorn MJB, von Deimling A, Westphal M, Soffietti R, Reifenberger G, Wick W
Nat Rev Clin Oncol 2021 Mar;18(3):170-186. Epub 2020 Dec 8 doi: 10.1038/s41571-020-00447-z. PMID: 33293629Free PMC Article
Immunotherapy for glioma: Current management and future application.
Xu S, Tang L, Li X, Fan F, Liu Z
Cancer Lett 2020 Apr 28;476:1-12. Epub 2020 Feb 7 doi: 10.1016/j.canlet.2020.02.002. PMID: 32044356
The Diagnosis and Management of Neurofibromatosis Type 1.
Ly KI, Blakeley JO
Med Clin North Am 2019 Nov;103(6):1035-1054. doi: 10.1016/j.mcna.2019.07.004. PMID: 31582003

Recent clinical studies

Etiology

Conventional MRI Features of Central Nervous System Embryonal Tumor, Not Otherwise Specified in Adults: Comparison with Glioblastoma.
Xiao X, Liu X, Liang W, Han LY, Li XD, Guo LJ, He WL, Liu XM, Zhou J, Cai Q, Xu YK, Tan XL, Wu YK
Acad Radiol 2022 Mar;29 Suppl 3:S44-S51. Epub 2021 Jan 25 doi: 10.1016/j.acra.2021.01.010. PMID: 33504445
Changes in Peripheral Blood Regulatory T Cells and IL-6 and IL-10 Levels Predict Response of Pediatric Medulloblastoma and Germ Cell Tumors With Residual or Disseminated Disease to Craniospinal Irradiation.
Song L, Wang S, Fang T, Qiu X, Wang X, Zhou X, Morse MA, Hobeika A, Wu W, Yang H, Ren J, Lyerly HK
Int J Radiat Oncol Biol Phys 2021 Oct 1;111(2):479-490. Epub 2021 May 8 doi: 10.1016/j.ijrobp.2021.04.041. PMID: 33974888
The case for DNA methylation based molecular profiling to improve diagnostic accuracy for central nervous system embryonal tumors (not otherwise specified) in adults.
Halliday GC, Junckerstorff RC, Bentel JM, Miles A, Jones DTW, Hovestadt V, Capper D, Endersby R, Cole CH, van Hagen T, Gottardo NG
J Clin Neurosci 2018 Jan;47:163-167. Epub 2017 Oct 6 doi: 10.1016/j.jocn.2017.09.013. PMID: 28993028

Diagnosis

Preoperative 11 C-Methionine PET-MRI in Pediatric Infratentorial Tumors.
Beuriat PA, Flaus A, Portefaix A, Szathmari A, Janier M, Hermier M, Lorthois-Ninou S, Scheiber C, Isal S, Costes N, Merida I, Lancelot S, Vasiljevic A, Leblond P, Faure Conter C, Saunier C, Kassai B, Vinchon M, Di Rocco F, Mottolese C
Clin Nucl Med 2024 May 1;49(5):381-386. Epub 2024 Mar 15 doi: 10.1097/RLU.0000000000005174. PMID: 38498623
Optical genome mapping identifies a novel pediatric embryonal tumor with a ZNF532::NUTM1 fusion.
Bornhorst M, Eze A Jr, Bhattacharya S, Putnam E, Almira-Suarez MI, Rossi C, Kambhampati M, Almalvez M, Barseghyan M, Del Risco N, Dotson D, Turner J, Myseros JS, Vilain E, Packer RJ, Nazarian J, Rood B, Barseghyan H
J Pathol 2023 Jul;260(3):329-338. Epub 2023 May 19 doi: 10.1002/path.6085. PMID: 37203791Free PMC Article
Conventional MRI Features of Central Nervous System Embryonal Tumor, Not Otherwise Specified in Adults: Comparison with Glioblastoma.
Xiao X, Liu X, Liang W, Han LY, Li XD, Guo LJ, He WL, Liu XM, Zhou J, Cai Q, Xu YK, Tan XL, Wu YK
Acad Radiol 2022 Mar;29 Suppl 3:S44-S51. Epub 2021 Jan 25 doi: 10.1016/j.acra.2021.01.010. PMID: 33504445
Hemorrhagic Suprasellar Central Nervous System Embryonal Tumor in an Adult: Uncommon Features of an Extremely Rare Neoplasm.
Piloni M, Gagliardi F, Bailo M, Barzaghi LR, Callea M, Terreni M, Falini A, Castellano A, Mortini P
J Neurol Surg A Cent Eur Neurosurg 2022 Jan;83(1):89-98. Epub 2021 Jan 21 doi: 10.1055/s-0040-1721022. PMID: 33477185
Changes in Peripheral Blood Regulatory T Cells and IL-6 and IL-10 Levels Predict Response of Pediatric Medulloblastoma and Germ Cell Tumors With Residual or Disseminated Disease to Craniospinal Irradiation.
Song L, Wang S, Fang T, Qiu X, Wang X, Zhou X, Morse MA, Hobeika A, Wu W, Yang H, Ren J, Lyerly HK
Int J Radiat Oncol Biol Phys 2021 Oct 1;111(2):479-490. Epub 2021 May 8 doi: 10.1016/j.ijrobp.2021.04.041. PMID: 33974888

Therapy

Extramedullary Hematopoiesis in the Dura Mater During Treatment of a CNS Embryonal Tumor.
Ganapathi SS, Havard ME, Ferguson M, Cole BL, Millard NE, Vitanza NA
J Pediatr Hematol Oncol 2021 Nov 1;43(8):e1217-e1219. doi: 10.1097/MPH.0000000000001962. PMID: 33031159

Prognosis

Changes in Peripheral Blood Regulatory T Cells and IL-6 and IL-10 Levels Predict Response of Pediatric Medulloblastoma and Germ Cell Tumors With Residual or Disseminated Disease to Craniospinal Irradiation.
Song L, Wang S, Fang T, Qiu X, Wang X, Zhou X, Morse MA, Hobeika A, Wu W, Yang H, Ren J, Lyerly HK
Int J Radiat Oncol Biol Phys 2021 Oct 1;111(2):479-490. Epub 2021 May 8 doi: 10.1016/j.ijrobp.2021.04.041. PMID: 33974888
Extramedullary Hematopoiesis in the Dura Mater During Treatment of a CNS Embryonal Tumor.
Ganapathi SS, Havard ME, Ferguson M, Cole BL, Millard NE, Vitanza NA
J Pediatr Hematol Oncol 2021 Nov 1;43(8):e1217-e1219. doi: 10.1097/MPH.0000000000001962. PMID: 33031159

Clinical prediction guides

Changes in Peripheral Blood Regulatory T Cells and IL-6 and IL-10 Levels Predict Response of Pediatric Medulloblastoma and Germ Cell Tumors With Residual or Disseminated Disease to Craniospinal Irradiation.
Song L, Wang S, Fang T, Qiu X, Wang X, Zhou X, Morse MA, Hobeika A, Wu W, Yang H, Ren J, Lyerly HK
Int J Radiat Oncol Biol Phys 2021 Oct 1;111(2):479-490. Epub 2021 May 8 doi: 10.1016/j.ijrobp.2021.04.041. PMID: 33974888

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