Article Figures & Data
Figures
- Fig 1.
Schematic representation of the external surface of cerebral hemispheres (A) shows the locations of hemispheric tumors, with the sizes of the shaded circles proportional to the number of tumors in each lobe. A midline sagittal section of the brain (B) shows pineal and nonpineal tumors, with pie diagrams representing the subgroups.
- Fig 2.
Kaplan-Meier curves show event-free survival distributions by tumor size (A), margins (B), presence of edema (C), and percentage enhancement (D) for all patients (n = 56).
- Fig 3.
(A and B), A 15-year-old girl with a molecular diagnosis of pineoblastoma. Sagittal T1-weighted postcontrast (A) and axial diffusion-weighted (B) MR images demonstrate a mass centered in the pineal region with diffuse heterogeneous enhancement, small cystic foci, and diffusion restriction. (C and D), A 9-year-old girl with a molecular diagnosis of ATRT_MYC. Sagittal T1-weighted postcontrast (C) and axial diffusion-weighted (D) images demonstrate a similar mass centered in the pineal region with diffuse heterogeneous enhancement, small cystic foci, and diffusion restriction. Note the similarities in imaging appearance between the 2 examples.
- Fig 4.
Sagittal T1-weighted postcontrast (A) and sagittal high-resolution balanced steady-state gradient-echo (B) images from 2 different patients with molecularly proved pineoblastomas demonstrating a tail-like aqueductal extension (white arrows).
- Fig 5.
(A and B), A 4-year-old girl with a molecular diagnosis of high-grade glioma (GBM_MYCN). Axial DWI (A) and sagittal postcontrast T1-weighted (B) images demonstrate a large mass centered in the left frontal lobe with prominent necrotic/cystic areas centrally and diffusion restriction and moderate heterogeneous enhancement of the solid component. (C and D), A 4-year-old boy with a molecular diagnosis of ependymoma (EP_RELA). Axial DWI (C) and sagittal postcontrast T1-weighted (D) images demonstrate a large mass centered deep in the left hemisphere with prominent necrotic/cystic areas centrally and diffusion restriction and moderate heterogeneous enhancement of the solid component. Note the similarities in age and imaging appearance between these 2 patients with different molecular diagnoses.
- Fig 6.
Two different patients with a molecular diagnosis of ET. (A and B), A 10-year-old girl with axial T2-weighted (A) and axial postcontrast T1-weighted (B) images has a large mass centered in the left deep nuclei with a prominent cystic component and moderate enhancement of the solid component. The tumor subclass was CNS_NB_FOXR2. Please note the similarities with high-grade glioma and ependymoma illustrated in Fig 5. (C and D), A 5-year-old girl with axial T2-weighted (C) and sagittal postcontrast T1-weighted (D) images has a large solid mass centered in the right lateral ventricle with minimal-to-no enhancement. The tumor subclass was ETMR. Of note, both of these tumors demonstrated diffusion restriction (not shown).
Tables
- Table 1:
Molecular diagnoses for tumors with both imaging and methylation profiles available (n = 56)a
PBL/ET No. Non-ET No. PBL 27 GBM_G34d 8 CNS_NB_FOXR2b 3 GBM_MYCNd 5 ETMRb 1 DMG_K27d 2 HGNET_MN1b 1 GBM_MIDd 2 MB_WNTb 1 EP_RELA 2 CNS ET, NOSb 1 ATRT_SHHc 2 ATRT_MYCc 1 Total 37 19 Note:—CNS_NB_FOXR2 indicates CNS neuroblastoma with FOXR2 activation; HGNET_MN1, CNS high-grade neuroepithelial tumor with MN1 alteration; MB_WNT: medulloblastoma with wingless (Wnt) activation; ATRT_SHH, atypical teratoid/rhabdoid tumor with sonic hedgehog (shh) activation; ATRT_MYC, atypical teratoid/rhabdoid tumor, subclass MYC; GBM_G34, glioblastoma, Isocitrate dehydrogenase (IDH) wild-type, H3.3 G34 mutant; GBM_MYCN, glioblastoma, IDH wild-type, subclass MYCN; DMG_K27, diffuse midline glioma H3K27M mutant; GBM-MID, glioblastoma, IDH wild-type, subclass midline; EP-RELA, ependymoma with positive RELA fusion.
↵a For further analysis, the following have been combined into single groups:
↵b As ET, other.
↵c As ATRT.
↵d As HGG.
Pineal Nonpineal PBL 26 1 ET, other 0 7 ATRT 1 2 HGG 1 16 EP 0 2 Total 28 28 Group P Value PBL/ET Non-ET All Patients No. % No. % No. % Size (cm) <.001 Median 3.6 – 6.2 – 4.3 – Minimum 1.1 – 2.7 – 1.1 – Maximum 9.1 – 9.3 – 9.3 – % Enhancement .17a, .80b None 1 2.7 0 0 1 1.8 0–25 3 8.1 3 15.8 6 10.7 25–75 6 16.2 7 36.8 13 23.2 >75 27 73.0 9 47.4 36 64.3 Margins <.001 Well-defined 37 100.0 13 68.4 50 89.3 Ill-defined 0 0 6 31.6 6 10.7 Presence of edema <.001c Absent 32 86.5 5 26.3 37 66.1 <2 cm from tumor margin 4 10.8 13 68.4 17 30.4 >2 cm from tumor margin 1 2.7 1 5.3 2 3.6 Presence of cyst/necrosis .22 Absent 12 32.4 3 15.8 15 26.8 Present 25 67.6 16 84.2 41 73.2 Presence of calcification or hemorrhage .26 Absent 16 43.2 5 26.3 21 37.5 Present 21 56.8 14 73.7 35 62.5 DWI – Bright 28 75.7 15 78.9 43 76.8 Dark 1 2.7 0 0 1 1.8 Intermediate 5 13.5 3 15.8 8 14.3 Artifact or not available 3 8.1 1 5.3 4 7.1 Metastasis – Intracranial 1 2.7 0 0 1 1.8 Spinal 5 13.5 0 0 5 8.9 Intracranial and spinal 6 16.2 0 0 6 10.7 None 25 67.6 19 100.0 44 78.6 All patients 37 100.0 19 100.0 56 100.0 Note:—– indicates no data available.
a Comparison of none versus 0%–25% versus 25%–75% versus >75%.
b Comparison of >75% versus ≤75%.
c Comparison of absent versus present.
Group P Value ET Non-ET All Patients No. % No. % No. % Size (cm) .95 Median 5.7 – 6.2 – 6.1 – Minimum 3.6 – 2.7 – 2.7 – Maximum 9.1 – 9.3 – 9.3 – % Enhancement .68a None 0 0 0 0 0 0 0–25 2 20 3 15.8 5 17.2 25–75 5 50 7 36.8 12 41.4 >75 3 30 9 47.4 12 41.4 Margins .068 Well-defined 10 100.0 13 68.4 23 79.3 Ill-defined 0 0 6 31.6 6 20.7 Presence of edema .24b Absent 5 50 5 26.3 10 34.5 <2 cm from tumor margin 4 40 13 68.4 17 58.6 >2 cm from tumor margin 1 10 1 5.3 2 6.9 Presence of cyst/necrosis .53 Absent 0 0 3 15.8 3 10.3 Present 10 100 16 84.2 26 89.7 Presence of calcification or hemorrhage .11 Absent 6 60 5 26.3 11 37.9 Present 4 40 14 73.7 18 62.1 DWI – Bright 9 90 15 78.9 24 82.8 Dark 0 0 0 0 0 0 Intermediate 0 0 3 15.8 3 10.3 Artifact or not available 1 10 1 5.3 2 6.9 Metastasis Intracranial 0 0 0 0 0 0 Spinal 0 0 0 0 0 0 Intracranial and spinal 0 0 0 0 0 0 None 10 100 19 100.0 29 100 All patients 10 100.0 19 100.0 29 100.0 Note:— – indicates no data available.
a Comparison of none versus 0%–25% versus 25%–75% versus >75%.
b Comparison of absent versus present.
