Pediatric-type diffuse low-grade glioma

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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.

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. ↑ 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.
  2. ↑ 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/.
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