Polymorphous low grade neuroepithelial tumor of the young

MedGen UID:: 1792771
•Concept ID:: C5556330
•: Neoplastic Process

Synonym:	Polymorphous low grade neuroepithelial tumour of the young

Monarch Initiative:	MONDO:0858959

Definition

A low-grade cerebral tumor associated with seizures and in many cases refractory epilepsy. It usually occurs in the second and third decades of life. It is characterized by the presence of oligodendroglioma-like components. It may also contain astrocytic components. MAPK pathway-activating genetic alterations play a role in the development of this tumor. Causative gene alterations include mutations resulting in the expression of BRAF p.V600E and gene fusions involving FGFR2 or FGFR3 genes. IDH gene mutations and 1p/19q codeletion are not present. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVPolymorphous low grade neuroepithelial tumor of the young

Disease
- Disorder by Site
  - Disorder of nervous system
    - Disorder of the central nervous system
      - Neoplasm of the central nervous system
        Neuronal/glioneuronal neoplasm of the central nervous system
        Polymorphous low grade neuroepithelial tumor of the young
        Recurrent Polymorphous Low Grade Neuroepithelial Tumor of the Young

Recent clinical studies

Etiology

Histological features in pediatric central nervous system tumors with FGFR alterations.

Gilani A, Davies K, Kleinschmidt-DeMasters B
Folia Neuropathol 2020;58(4):347-356. doi: 10.5114/fn.2020.102437. PMID: 33480239

Plenty of calcification: imaging characterization of polymorphous low-grade neuroepithelial tumor of the young.

Johnson DR, Giannini C, Jenkins RB, Kim DK, Kaufmann TJ
Neuroradiology 2019 Nov;61(11):1327-1332. Epub 2019 Aug 9 doi: 10.1007/s00234-019-02269-y. PMID: 31396664

See all (2)

Diagnosis

Neuroradiological features of the polymorphous low-grade neuroepithelial tumor of the young: five new cases with a systematic review of the literature.

Kurokawa M, Kurokawa R, Capizzano AA, Baba A, Ota Y, Pinarbasi E, Johnson T, Srinivasan A, Moritani T
Neuroradiology 2022 Jun;64(6):1255-1264. Epub 2022 Jan 10 doi: 10.1007/s00234-021-02879-5. PMID: 35001164

Polymorphous low-grade neuroepithelial tumor of the young: case report and review focus on the radiological features and genetic alterations.

Chen Y, Tian T, Guo X, Zhang F, Fan M, Jin H, Liu D
BMC Neurol 2020 Apr 6;20(1):123. doi: 10.1186/s12883-020-01679-3. PMID: 32252664 Free PMC Article

Polymorphous Low-Grade Neuroepithelial Tumor of the Young: A Case Report with Genomic Findings.

Gupta VR, Giller C, Kolhe R, Forseen SE, Sharma S
World Neurosurg 2019 Dec;132:347-355. Epub 2019 Sep 11 doi: 10.1016/j.wneu.2019.08.221. PMID: 31520766

Plenty of calcification: imaging characterization of polymorphous low-grade neuroepithelial tumor of the young.

Johnson DR, Giannini C, Jenkins RB, Kim DK, Kaufmann TJ
Neuroradiology 2019 Nov;61(11):1327-1332. Epub 2019 Aug 9 doi: 10.1007/s00234-019-02269-y. PMID: 31396664

Case Report of Rarely Described Polymorphous Low-Grade Neuroepithelial Tumor of the Young and Comparison with Oligodendroglioma.

Sumdani H, Shahbuddin Z, Harper G, Hamilton L
World Neurosurg 2019 Jul;127:47-51. Epub 2019 Mar 26 doi: 10.1016/j.wneu.2019.03.181. PMID: 30926558

See all (7)

Prognosis

Pediatric and elderly polymorphous low-grade neuroepithelial tumor of the young: Typical and unusual case reports and literature review.

Furuta T, Moritsubo M, Muta H, Shimamoto H, Ohshima K, Sugita Y
Neuropathology 2023 Aug;43(4):319-325. Epub 2022 Dec 22 doi: 10.1111/neup.12889. PMID: 36545913

Histological features in pediatric central nervous system tumors with FGFR alterations.

Gilani A, Davies K, Kleinschmidt-DeMasters B
Folia Neuropathol 2020;58(4):347-356. doi: 10.5114/fn.2020.102437. PMID: 33480239

See all (2)