General Information of Disease (ID: DISJYLBY)

Disease Name Nerve sheath neoplasm
Synonyms tumour of nerve sheath; tumor of nerve sheath; peripheral nerve sheath neoplasm; nerve sheath tumour; nerve sheath tumor; nerve sheath neoplasm; neoplasm of the nerve sheath; neoplasm of nerve sheath
Definition
A benign or malignant neoplasm arising from the perineural cells in the sheaths surrounding the nerves. Representative examples include neurofibroma, schwannoma, and malignant peripheral nerve sheath tumor.
Disease Hierarchy
DISG0QYN: Peripheral nervous system tumour
DISTBY9Z: Tumour
DIS5RPEH: Glioma
DISJYLBY: Nerve sheath neoplasm
Disease Identifiers
MONDO ID
MONDO_0002547
MESH ID
D018317
UMLS CUI
C0206727
MedGen ID
64639
SNOMED CT ID
115242003

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 2 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
CDKN2A TTFTWQ8 Limited Biomarker [1]
EED TTFNJ4R Limited Biomarker [1]
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This Disease Is Related to 3 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
SUZ12 OT655XF8 Limited Biomarker [1]
NF1 OTC29NHH Strong Biomarker [1]
SOX10 OTF25ULQ Strong Biomarker [2]
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References

1 PRC2 is recurrently inactivated through EED or SUZ12 loss in malignant peripheral nerve sheath tumors.Nat Genet. 2014 Nov;46(11):1227-32. doi: 10.1038/ng.3095. Epub 2014 Sep 21.
2 Benign epithelioid peripheral nerve sheath tumour arising in the rectum: A cytological and histological description.Diagn Cytopathol. 2019 Aug;47(8):817-820. doi: 10.1002/dc.24185. Epub 2019 Apr 8.