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Visual Pathway Medulloepithelioma

MedGen UID:
1735597
•Concept ID:
C5421217
•
Neoplastic Process

Definition

A very rare, benign or malignant embryonal neoplasm affecting the visual pathway during childhood. It arises from primitive medullary epithelium. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVisual Pathway Medulloepithelioma

Professional guidelines

PubMed

Current and future strategies in radiotherapy of childhood low-grade glioma of the brain. Part I: Treatment modalities of radiation therapy.
Kortmann RD, Timmermann B, Taylor RE, Scarzello G, Plasswilm L, Paulsen F, Jeremic B, Gnekow AK, Dieckmann K, Kay S, Bamberg M
Strahlenther Onkol 2003 Aug;179(8):509-20. doi: 10.1007/s00066-003-9104-9. PMID: 14509949

Recent clinical studies

Etiology

Presenting symptoms and time to diagnosis for Pediatric Central Nervous System Tumors in Qatar: a report from Pediatric Neuro-Oncology Service in Qatar.
Maaz AUR, Yousif T, Saleh A, Pople I, Al-Kharazi K, Al-Rayahi J, Elkum N, Malik M
Childs Nerv Syst 2021 Feb;37(2):465-474. Epub 2020 Jul 24 doi: 10.1007/s00381-020-04815-z. PMID: 32710251Free PMC Article
A microRNA-1280/JAG2 network comprises a novel biological target in high-risk medulloblastoma.
Wang F, Remke M, Bhat K, Wong ET, Zhou S, Ramaswamy V, Dubuc A, Fonkem E, Salem S, Zhang H, Hsieh TC, O'Rourke ST, Wu L, Li DW, Hawkins C, Kohane IS, Wu JM, Wu M, Taylor MD, Wu E
Oncotarget 2015 Feb 20;6(5):2709-24. doi: 10.18632/oncotarget.2779. PMID: 25576913Free PMC Article
Current and future strategies in radiotherapy of childhood low-grade glioma of the brain. Part I: Treatment modalities of radiation therapy.
Kortmann RD, Timmermann B, Taylor RE, Scarzello G, Plasswilm L, Paulsen F, Jeremic B, Gnekow AK, Dieckmann K, Kay S, Bamberg M
Strahlenther Onkol 2003 Aug;179(8):509-20. doi: 10.1007/s00066-003-9104-9. PMID: 14509949
Visual evoked potentials in tumors from orbita to occipital lobe in childhood.
Wenzel D, Brandl U, Beck JD, Cedzich C, Albert F
Neurosurg Rev 1988;11(3-4):279-86. doi: 10.1007/BF01741423. PMID: 3251171

Diagnosis

Presenting symptoms and time to diagnosis for Pediatric Central Nervous System Tumors in Qatar: a report from Pediatric Neuro-Oncology Service in Qatar.
Maaz AUR, Yousif T, Saleh A, Pople I, Al-Kharazi K, Al-Rayahi J, Elkum N, Malik M
Childs Nerv Syst 2021 Feb;37(2):465-474. Epub 2020 Jul 24 doi: 10.1007/s00381-020-04815-z. PMID: 32710251Free PMC Article
A microRNA-1280/JAG2 network comprises a novel biological target in high-risk medulloblastoma.
Wang F, Remke M, Bhat K, Wong ET, Zhou S, Ramaswamy V, Dubuc A, Fonkem E, Salem S, Zhang H, Hsieh TC, O'Rourke ST, Wu L, Li DW, Hawkins C, Kohane IS, Wu JM, Wu M, Taylor MD, Wu E
Oncotarget 2015 Feb 20;6(5):2709-24. doi: 10.18632/oncotarget.2779. PMID: 25576913Free PMC Article
Green tea polyphenols enhance sodium nitroprusside-induced neurotoxicity in human neuroblastoma SH-SY5Y cells.
Zhang Y, Zhao B
J Neurochem 2003 Sep;86(5):1189-200. doi: 10.1046/j.1471-4159.2003.01928.x. PMID: 12911627
Visual evoked potentials in tumors from orbita to occipital lobe in childhood.
Wenzel D, Brandl U, Beck JD, Cedzich C, Albert F
Neurosurg Rev 1988;11(3-4):279-86. doi: 10.1007/BF01741423. PMID: 3251171
Tumors of the optic nerve.
Eggers H, Jakobiec FA, Jones IS
Doc Ophthalmol 1976 Apr 28;41(1):43-128. doi: 10.1007/BF00144713. PMID: 773617

Therapy

Novel GLP-1R/GIPR co-agonist "twincretin" is neuroprotective in cell and rodent models of mild traumatic brain injury.
Tamargo IA, Bader M, Li Y, Yu SJ, Wang Y, Talbot K, DiMarchi RD, Pick CG, Greig NH
Exp Neurol 2017 Feb;288:176-186. Epub 2016 Nov 11 doi: 10.1016/j.expneurol.2016.11.005. PMID: 27845037Free PMC Article
The nuclear factor erythroid 2-like 2 activator, tert-butylhydroquinone, improves cognitive performance in mice after mild traumatic brain injury.
Saykally JN, Rachmany L, Hatic H, Shaer A, Rubovitch V, Pick CG, Citron BA
Neuroscience 2012 Oct 25;223:305-14. Epub 2012 Aug 10 doi: 10.1016/j.neuroscience.2012.07.070. PMID: 22890082Free PMC Article
Resveratrol inhibits tumor growth of human neuroblastoma and mediates apoptosis by directly targeting mitochondria.
van Ginkel PR, Sareen D, Subramanian L, Walker Q, Darjatmoko SR, Lindstrom MJ, Kulkarni A, Albert DM, Polans AS
Clin Cancer Res 2007 Sep 1;13(17):5162-9. doi: 10.1158/1078-0432.CCR-07-0347. PMID: 17785572
Current and future strategies in radiotherapy of childhood low-grade glioma of the brain. Part I: Treatment modalities of radiation therapy.
Kortmann RD, Timmermann B, Taylor RE, Scarzello G, Plasswilm L, Paulsen F, Jeremic B, Gnekow AK, Dieckmann K, Kay S, Bamberg M
Strahlenther Onkol 2003 Aug;179(8):509-20. doi: 10.1007/s00066-003-9104-9. PMID: 14509949
Current therapy in childhood brain tumors.
Cohen ME, Duffner PK
Neurol Clin 1985 Feb;3(1):147-64. PMID: 3887125

Prognosis

A microRNA-1280/JAG2 network comprises a novel biological target in high-risk medulloblastoma.
Wang F, Remke M, Bhat K, Wong ET, Zhou S, Ramaswamy V, Dubuc A, Fonkem E, Salem S, Zhang H, Hsieh TC, O'Rourke ST, Wu L, Li DW, Hawkins C, Kohane IS, Wu JM, Wu M, Taylor MD, Wu E
Oncotarget 2015 Feb 20;6(5):2709-24. doi: 10.18632/oncotarget.2779. PMID: 25576913Free PMC Article
OTX2 directly activates cell cycle genes and inhibits differentiation in medulloblastoma cells.
Bunt J, Hasselt NE, Zwijnenburg DA, Hamdi M, Koster J, Versteeg R, Kool M
Int J Cancer 2012 Jul 15;131(2):E21-32. Epub 2011 Nov 8 doi: 10.1002/ijc.26474. PMID: 21964830
Current and future strategies in radiotherapy of childhood low-grade glioma of the brain. Part I: Treatment modalities of radiation therapy.
Kortmann RD, Timmermann B, Taylor RE, Scarzello G, Plasswilm L, Paulsen F, Jeremic B, Gnekow AK, Dieckmann K, Kay S, Bamberg M
Strahlenther Onkol 2003 Aug;179(8):509-20. doi: 10.1007/s00066-003-9104-9. PMID: 14509949
Visual impairment associated with mutism after posterior fossa surgery in children.
Liu GT, Phillips PC, Molloy PT, Needle MN, Galetta SL, Balcer LJ, Schut L, Duhaime AC, Sutton LN
Neurosurgery 1998 Feb;42(2):253-6; discussion 256-7. doi: 10.1097/00006123-199802000-00027. PMID: 9482175
Visual evoked potentials in tumors from orbita to occipital lobe in childhood.
Wenzel D, Brandl U, Beck JD, Cedzich C, Albert F
Neurosurg Rev 1988;11(3-4):279-86. doi: 10.1007/BF01741423. PMID: 3251171

Clinical prediction guides

Resveratrol inhibits tumor growth of human neuroblastoma and mediates apoptosis by directly targeting mitochondria.
van Ginkel PR, Sareen D, Subramanian L, Walker Q, Darjatmoko SR, Lindstrom MJ, Kulkarni A, Albert DM, Polans AS
Clin Cancer Res 2007 Sep 1;13(17):5162-9. doi: 10.1158/1078-0432.CCR-07-0347. PMID: 17785572
Teneurin 2 is expressed by the neurons of the thalamofugal visual system in situ and promotes homophilic cell-cell adhesion in vitro.
Rubin BP, Tucker RP, Brown-Luedi M, Martin D, Chiquet-Ehrismann R
Development 2002 Oct;129(20):4697-705. doi: 10.1242/dev.129.20.4697. PMID: 12361962
retSDR1, a short-chain retinol dehydrogenase/reductase, is retinoic acid-inducible and frequently deleted in human neuroblastoma cell lines.
Cerignoli F, Guo X, Cardinali B, Rinaldi C, Casaletto J, Frati L, Screpanti I, Gudas LJ, Gulino A, Thiele CJ, Giannini G
Cancer Res 2002 Feb 15;62(4):1196-204. PMID: 11861404

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