Details of Disease

General Information of Disease (ID: DISCYKLP)

• Disease Name: Neuroepithelial neoplasm
• Synonyms: primary neuroepithelial tumour; primary neuroepithelial tumor; neoplasm of neuroepithelial tissue; neuroepithelial tumors; tumor of neuroepithelium; tumor of the neuroepithelium; neuroepithelial tumour; neuroepithelial tissue tumour; neoplasm of the neuroepithelium; tumour of neuroepithelium; neuroepithelial neoplasm; neuroepithelial tumor; neuroepithelial neoplasms; tumour of neuroepithelial tissue; tumour of the neuroepithelium; neoplasm of neuroepithelium; neuroepithelial tissue tumor; tumor of neuroepithelial tissue; neuroepithelial tumours; neuroepithelial tissue neoplasm
• Definition: A neoplasm of the nervous system that arises from the neuroepithelial tissues. Representative examples include astrocytic tumors, oligodendroglial tumors, ependymal tumors, and primitive neuroectodermal tumors.|Editor note consider adding grouping class for Neuroepithelial, Perineurial, and Schwann Cell Neoplasm
• Disease Hierarchy:
  DIS141UP: Nervous system neoplasm
  DISTBY9Z: Tumour
  DISCYKLP: Neuroepithelial neoplasm
• Disease Identifiers:
  MONDO ID: MONDO_0021193
  MESH ID: D018302
  UMLS CUI: C0206715
  MedGen ID: 60215
  HPO ID: HP:0030063
  SNOMED CT ID: 115241005

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 1 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
TOP2B	TT4NVEM	Strong	Altered Expression	[1]

This Disease Is Related to 5 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
BCOR	OTG013AX	Limited	Genetic Variation	[2]
OTP	OTS0JN6Y	moderate	Altered Expression	[3]
H3-3B	OT9XHQ3C	Strong	Genetic Variation	[4]
MN1	OTVQR4R9	Strong	Biomarker	[5]
NELL2	OTS4MJZ7	Strong	Altered Expression	[6]

References

• [1] Enhanced expression of DNA topoisomerase II genes in human medulloblastoma and its possible association with etoposide sensitivity.J Neurooncol. 2007 Sep;84(2):119-29. doi: 10.1007/s11060-007-9360-0. Epub 2007 Mar 15.
• [2] High-grade neuroepithelial tumor with BCOR exon 15 internal tandem duplication-a comprehensive clinical, radiographic, pathologic, and genomic analysis.Brain Pathol. 2020 Jan;30(1):46-62. doi: 10.1111/bpa.12747. Epub 2019 Jun 10.
• [3] Expression of Insulinoma-Associated Protein 1 (INSM1) and Orthopedia Homeobox (OTP) in Tumors with Neuroendocrine Differentiation at Rare Sites.Endocr Pathol. 2019 Mar;30(1):35-42. doi: 10.1007/s12022-018-9559-y.
• [4] H3F3A-G34R mutant high grade neuroepithelial neoplasms with glial and dysplastic ganglion cell components.Acta Neuropathol Commun. 2019 May 20;7(1):78. doi: 10.1186/s40478-019-0731-5.
• [5] Multimodal molecular analysis of astroblastoma enables reclassification of most cases into more specific molecular entities.Brain Pathol. 2018 Mar;28(2):192-202. doi: 10.1111/bpa.12561. Epub 2017 Oct 27.
• [6] Brain specific human genes, NELL1 and NELL2, are predominantly expressed in neuroblastoma and other embryonal neuroepithelial tumors.Neurol Med Chir (Tokyo). 2001 Dec;41(12):582-8; discussion 589. doi: 10.2176/nmc.41.582.