A single-center study of the clinicopathologic correlates of gliomas with a MYB or MYBL1 alteration
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Molecular alterations are increasingly being used to distinguish tumors with similar histopathologic features but distinct clinical characteristics. Among IDH-wildtype and H3-wildtype (IDH-wt/H3-wt) diffuse gliomas is an important, though uncommon, group of tumors presenting in children and young adults. These demonstrate an indolent clinical behavior, unlike IDH-mutant diffuse gliomas and the IDH-wt/H3-wt diffuse gliomas of later life [2, 3]. This group of ‘pediatric-type’ diffuse gliomas has a relatively restricted range of genetic alterations, which are distinct from those in ‘adult-type’ IDH-wt/H3-wt diffuse gliomas and involve FGFR1, BRAF, MYB and MYBL1 [2, 4]. Following the 2016 update to the World Health Organization (WHO) classification of CNS tumors, entities delineated by combinations of histopathologic and genetic characteristics and with distinctive clinical profiles have been presented in a series of papers from the cIMPACT-NOW group. These new entities include the...
- 3.Ellison DW, Hawkins C, Jones DTW, Onar-Thomas A, Pfister SM, Reifenberger G et al (2019) cIMPACT-NOW update 4: diffuse gliomas characterized by MYB, MYBL1, or FGFR1 alterations or BRAF(V600E) mutation. Acta Neuropathol 137:683–687. https://doi.org/10.1007/s00401-019-01987-0 CrossRefPubMedPubMedCentralGoogle Scholar
- 4.Qaddoumi I, Orisme W, Wen J, Santiago T, Gupta K, Dalton JD et al (2016) Genetic alterations in uncommon low-grade neuroepithelial tumors: BRAF, FGFR1, and MYB mutations occur at high frequency and align with morphology. Acta Neuropathol 131:833–845. https://doi.org/10.1007/s00401-016-1539-z CrossRefPubMedPubMedCentralGoogle Scholar