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Research ArticleBRAIN TUMOR IMAGING

T2-FLAIR Mismatch: An Imaging Biomarker for Children’s MYB/MYBL1–Altered Diffuse Astrocytoma or Angiocentric Glioma

E.A. van Maren, J.W. Dankbaar, P. Wesseling, S. Plasschaert, A. Muhlebner, E.W. Hoving, P.A. Robe, T.J. Snijders, R. Hoogendijk, M.E.G. Kranendonk and M.H. Lequin
American Journal of Neuroradiology May 2024, DOI: https://doi.org/10.3174/ajnr.A8203
E.A. van Maren
aFrom the Department of Radiology and Nuclear Medicine (E.A.v.M., J.W.D., M.H.L.), University Medical Center Utrecht, Utrecht, the Netherlands
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J.W. Dankbaar
aFrom the Department of Radiology and Nuclear Medicine (E.A.v.M., J.W.D., M.H.L.), University Medical Center Utrecht, Utrecht, the Netherlands
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P. Wesseling
bPrincess Máxima Center for Pediatric Oncology (P.W., S.P., E.W.H., R.H., M.E.G.K., M.H.L.), Utrecht, the Netherlands
cDepartment of Pathology (P.W.), Amsterdam University Medical Centers/VU Medical Center, Amsterdam, the Netherlands
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S. Plasschaert
bPrincess Máxima Center for Pediatric Oncology (P.W., S.P., E.W.H., R.H., M.E.G.K., M.H.L.), Utrecht, the Netherlands
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A. Muhlebner
dDepartment of Pathology (A.M.), University Medical Center Utrecht, Utrecht, the Netherlands
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E.W. Hoving
bPrincess Máxima Center for Pediatric Oncology (P.W., S.P., E.W.H., R.H., M.E.G.K., M.H.L.), Utrecht, the Netherlands
eDepartment of Neurosurgery (E.W.H., P.A.R.), University Medical Center Utrecht, Utrecht, the Netherlands
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P.A. Robe
eDepartment of Neurosurgery (E.W.H., P.A.R.), University Medical Center Utrecht, Utrecht, the Netherlands
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T.J. Snijders
fDepartment of Neurology (T.J.S.), UMC Utrecht Brain Center, University Medical Center Utrecht, Utrecht, the Netherlands
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R. Hoogendijk
bPrincess Máxima Center for Pediatric Oncology (P.W., S.P., E.W.H., R.H., M.E.G.K., M.H.L.), Utrecht, the Netherlands
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M.E.G. Kranendonk
bPrincess Máxima Center for Pediatric Oncology (P.W., S.P., E.W.H., R.H., M.E.G.K., M.H.L.), Utrecht, the Netherlands
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M.H. Lequin
aFrom the Department of Radiology and Nuclear Medicine (E.A.v.M., J.W.D., M.H.L.), University Medical Center Utrecht, Utrecht, the Netherlands
bPrincess Máxima Center for Pediatric Oncology (P.W., S.P., E.W.H., R.H., M.E.G.K., M.H.L.), Utrecht, the Netherlands
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  • FIG 1.
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    FIG 1.

    T2-FLAIR mismatch sign and area in MYB/MYBL1–altered diffuse astrocytoma and IDH-mutant glioma, T2- and T2-FLAIR-weighted sequences. A and B, MYB/MYBL1–altered diffuse astrocytoma left parietal lobe, mismatch sign. C and D, MYB/MYBL–altered diffuse astrocytoma left temporal lobe, mismatch area. E and F, IDH-mutant astrocytoma right temporal lobe, mismatch sign. G and H, IDH-mutant astrocytoma left frontoparietal lobe, mismatch area.

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    FIG 2.

    A–D, Low-grade tumors, MYB/MYBL1–altered. A, T2-FLAIR mismatch and microcysts in an angiocentric glioma. B, T2-FLAIR mismatch and an enlarged intercellular space (EIS). C, T2-FLAIR mismatch without microcyst or EIS. D, No T2-FLAIR mismatch with EIS. B–D, All from diffuse astrocytomas, MYB/MYBL1–altered. E–H, Diffuse astrocytoma, IDH-mutant. E, T2-FLAIR mismatch and microcysts in grade 3 astrocytoma. F, No T2-FLAIR mismatch with microcysts in grade 2 astrocytoma. G, No T2-FLAIR mismatch and no microcyst or EIS in grade 2 astrocytoma. H, No T2-FLAIR mismatch with EIS in grade 2 astrocytoma. Magnification, 50–100×; inset, 200–400×.

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    Table 2:

    Characteristics and results of the cases with astrocytoma, IDH-mutant, CNS WHO grades 2–4

    CaseAge (yr)SubgroupFusion/Methylation AnalysisT2-FLAIR Mismatch Sign (S) or Area (A)Histology
    MicrocystsEnlarged Intercellular Spaces
    1217.7IDH +, WHO 2IDH (R132H), ATRX, TP53+ (A)++
    139.2IDH +, WHO 2IDH (R132H), TP53–++
    1418.7IDH +, WHO 2IDH (R132H), ATRX, TP53+ (S)–+
    1514.0IDH +, WHO 2IDH (R132H), ATRX, TP53–++
    1610.6IDH +, WHO 2IDH (R132H), TP53––+
    174.0IDH +, WHO 2IDH (R132H), PIK3CA, NF1–+–
    1817.1IDH +, WHO 3IDH (R132H), PTEN, TP53–++
    1915.6IDH +, WHO 4IDH (R132H), TP53, MSH6, ATRX, CDKN2A, CIC.–
    • Note:—The + indicates presence; −, absence of the characteristic.

    • View popup
    Table 1:

    Characteristics and results of the cases with diffuse astrocytoma MYB/MYBL1–altered or angiocentric glioma, CNS WHO grade 1

    CaseAge (yr)SubgroupFusion/Methylation AnalysisT2-FLAIR Mismatch Sign (S) or Area (A)Histology
    MicrocystsEnlarged Intercellular Spaces
    11.1Diffuse Astro.MYBL1::MMP16+ (A)––
    23.2Diffuse Astro.MYBL1::STAU2-AS1+ (S)––
    39.7Diffuse Astro.MYBL1::PACRG+ (S)++
    49.0Angio-centric gliomaNo fusion analysisa+ (A)–+
    53.9Diffuse Astro.MYBL1::TOX+ (S)++
    611.4Diffuse Astro.No fusion analysis, diagnosis based on methylome profile+ (A)–+
    74.4Diffuse Astro.No fusion analysisa+ (A)–+
    815.9Angio-centric gliomaMYB::QKI–––
    95.7Diffuse Astro.No fusion analysisa––+
    109.6Diffuse Astro.No fusion analysisa––+
    118.2Angio-centric gliomaMYB::QKI+ (A)––
    • Note:—The + indicates presence; −, absence of the characteristic; Astro. astrocytoma.

    • ↵a In case no fusion analysis was available, the diagnosis of diffuse astrocytoma, MYB- or MYBL1–altered or angiocentric glioma was based on results of histology in combination with methylome profiling using the Infinium MethylationEPIC (850k) BeadChip (Illumina, San Diego, CA) methylation array.

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T2-FLAIR Mismatch: An Imaging Biomarker for Children’s MYB/MYBL1–Altered Diffuse Astrocytoma or Angiocentric Glioma
E.A. van Maren, J.W. Dankbaar, P. Wesseling, S. Plasschaert, A. Muhlebner, E.W. Hoving, P.A. Robe, T.J. Snijders, R. Hoogendijk, M.E.G. Kranendonk, M.H. Lequin
American Journal of Neuroradiology May 2024, DOI: 10.3174/ajnr.A8203
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E.A. van Maren, J.W. Dankbaar, P. Wesseling, S. Plasschaert, A. Muhlebner, E.W. Hoving, P.A. Robe, T.J. Snijders, R. Hoogendijk, M.E.G. Kranendonk, M.H. Lequin
T2-FLAIR Mismatch: An Imaging Biomarker for Children’s MYB/MYBL1–Altered Diffuse Astrocytoma or Angiocentric Glioma
American Journal of Neuroradiology May 2024, DOI: 10.3174/ajnr.A8203

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