Pediatric-type diffuse low-grade glioma
The category Pediatric-type diffuse low-grade glioma contains diffusely-growing brain tumours with specific molecular alterations belonging into the main group of Glioma. This category consists of mostly CNS WHO grade 1 tumors, observed in children and young-adults.
The fifth edition of CNS WHO classfication recognizes four distinct tumour diagnoses.
- Diffuse astrocytoma, MYB- or MYBL-altered.
- Angiocentric glioma.
- Polymorphous low-grade tumor of the young (PLNTY).
- Diffuse low-grade glioma, MAPK pathway-altered.
Polymorphous low-grade tumor of the young (PLNTY)
- Since 2021 official WHO entity.[1]
- Abbreviated PLNTY.
- Epilepsy-associated.
- Oligodendroglioma-like histologic features.
- Frequently CD34+ve.[2]
- FGFR3-TACC3 and FGFR2-CTNNA3 fusions.
Diffuse low-grade glioma, MAPK pathway-altered
- Since 2021 official WHO entity.[1]
- Astrocytic or oligodendroglial morphology.
- Mostly in the cerebral hemispheres.
- Commonly associated with epilepsy.
- Olig2-ve, GFAP variable, CD34 mostly absent.
- IDH and HF3FA mutations and CDKN2A deletions not compatible with this diagnosis.
- Mutations in BRAF (V600E), FGFR1 or duplication in FGFR1 TKD.
References
- ↑ 1.0 1.1 Bale TA, Rosenblum MK (2022). "The 2021 WHO Classification of Tumors of the Central Nervous System: An update on pediatric low-grade gliomas and glioneuronal tumors.". Brain Pathol: e13060. doi:10.1111/bpa.13060. PMID 35218102. https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=35218102.
- ↑ Huse JT, Snuderl M, Jones DT, Brathwaite CD, Altman N, Lavi E, Saffery R, Sexton-Oates A, Blumcke I, Capper D, Karajannis MA, Benayed R, Chavez L, Thomas C, Serrano J, Borsu L, Ladanyi M, Rosenblum MK (March 2017). "Polymorphous low-grade neuroepithelial tumor of the young (PLNTY): an epileptogenic neoplasm with oligodendroglioma-like components, aberrant CD34 expression, and genetic alterations involving the MAP kinase pathway". Acta Neuropathol 133 (3): 417–429. doi:10.1007/s00401-016-1639-9. PMC 5325850. PMID 27812792. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5325850/.