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

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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×.

Table 2:

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

Case	Age (yr)	Subgroup	Fusion/Methylation Analysis	T2-FLAIR Mismatch Sign (S) or Area (A)	Histology
Case	Age (yr)	Subgroup	Fusion/Methylation Analysis	T2-FLAIR Mismatch Sign (S) or Area (A)	Microcysts	Enlarged Intercellular Spaces
12	17.7	IDH +, WHO 2	IDH (R132H), ATRX, TP53	+ (A)	+	+
13	9.2	IDH +, WHO 2	IDH (R132H), TP53	–	+	+
14	18.7	IDH +, WHO 2	IDH (R132H), ATRX, TP53	+ (S)	–	+
15	14.0	IDH +, WHO 2	IDH (R132H), ATRX, TP53	–	+	+
16	10.6	IDH +, WHO 2	IDH (R132H), TP53	–	–	+
17	4.0	IDH +, WHO 2	IDH (R132H), PIK3CA, NF1	–	+	–
18	17.1	IDH +, WHO 3	IDH (R132H), PTEN, TP53	–	+	+
19	15.6	IDH +, WHO 4	IDH (R132H), TP53, MSH6, ATRX, CDKN2A, CIC.	–

Table 1:

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

Case	Age (yr)	Subgroup	Fusion/Methylation Analysis	T2-FLAIR Mismatch Sign (S) or Area (A)	Histology
Case	Age (yr)	Subgroup	Fusion/Methylation Analysis	T2-FLAIR Mismatch Sign (S) or Area (A)	Microcysts	Enlarged Intercellular Spaces
1	1.1	Diffuse Astro.	MYBL1::MMP16	+ (A)	–	–
2	3.2	Diffuse Astro.	MYBL1::STAU2-AS1	+ (S)	–	–
3	9.7	Diffuse Astro.	MYBL1::PACRG	+ (S)	+	+
4	9.0	Angio-centric glioma	No fusion analysis^{^a}	+ (A)	–	+
5	3.9	Diffuse Astro.	MYBL1::TOX	+ (S)	+	+
6	11.4	Diffuse Astro.	No fusion analysis, diagnosis based on methylome profile	+ (A)	–	+
7	4.4	Diffuse Astro.	No fusion analysis^{^a}	+ (A)	–	+
8	15.9	Angio-centric glioma	MYB::QKI	–	–	–
9	5.7	Diffuse Astro.	No fusion analysis^{^a}	–	–	+
10	9.6	Diffuse Astro.	No fusion analysis^{^a}	–	–	+
11	8.2	Angio-centric glioma	MYB::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.