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Research ArticlePediatrics

MRI Characteristics of Primary Tumors and Metastatic Lesions in Molecular Subgroups of Pediatric Medulloblastoma: A Single-Center Study

D. Mata-Mbemba, M. Zapotocky, S. Laughlin, M.D. Taylor, V. Ramaswamy and C. Raybaud
American Journal of Neuroradiology May 2018, 39 (5) 949-955; DOI: https://doi.org/10.3174/ajnr.A5578
D. Mata-Mbemba
aFrom the Department of Diagnostic Imaging (D.M.-M., S.L., C.R.)
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M. Zapotocky
bDivision of Neuro-oncology (M.Z., V.R.)
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S. Laughlin
aFrom the Department of Diagnostic Imaging (D.M.-M., S.L., C.R.)
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M.D. Taylor
cDepartment of Neurosurgery (M.D.T.), Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
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V. Ramaswamy
bDivision of Neuro-oncology (M.Z., V.R.)
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C. Raybaud
aFrom the Department of Diagnostic Imaging (D.M.-M., S.L., C.R.)
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Figures

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  • Fig 1.
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    Fig 1.

    Coronal postcontrast T1WI (A) showing SHH tumor within the fourth ventricle. The postoperative sagittal postcontrast T1 (B) confirmed that the tumor was arising from the flocculonodular lobe.

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    Fig 2.

    Multifocal (synchronous) SHH tumors at diagnosis. The multiple tumors on this axial postcontrast T1 are located in, and presumably originate within, the cerebellar cortex (A). In the second patient (B), there is 1 large fourth ventricular mass with an associated separate cortical mass posteriorly. In this case, the main tumor is lobulated and appears more like a conglomerate of multiple masses.

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    Fig 3.

    Metastatic group 3 tumor. In the first patient (A and B), the leptomeningeal “sugar coating” metastases demonstrate both diffusion restriction (A) and leptomeningeal enhancement on an axial postcontrast T1 image (B). In the second patient, the primary tumor appears smaller than its leptomeningeal suprasellar, avidly enhancing metastasis (C). An avidly enhancing nodular metastasis involving the cortex in the cerebellum posteriorly is also noted.

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    Fig 4.

    Metastatic group 4 tumors. In the first patient (A and B), the ependymal metastases seen in the anterior horns of the lateral ventricles and in the splenium of corpus callosum demonstrate diffusion restriction (B) without enhancement on contrast-enhanced T1 (arrows, B), while the leptomeningeal metastasis seen along the right temporal lobe demonstrates enhancement without appreciable diffusion restriction (arrowheads, B). In the second patient (C), the primary tumor demonstrates minimal enhancement on the contrast-enhanced T1 (arrowheads). The patient has a metastatic tumor in the suprasellar region with 2 components: One is ependymal in the infundibular recess and shows no enhancement (thin arrow), while the second is leptomeningeal and enhances strongly (thick arrow) like other metastasis in the posterior fossa, or supratentorially.

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    Fig 5.

    A recurrent group 4 tumor. A, The primary tumor demonstrates minimal postcontrast enhancement. Four years later, a recurrent tumor appeared along the lower margin of the postoperative cavity (arrows, B) as well as a metastasis in the infundibular recess and along the tuber cinereum (arrow, C), both of which are nonenhancing.

Tables

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    Table 1:

    Patient demographics with respect to molecular groups

    ParametersWNT (n = 15) (%)SHH (n = 26) (%)Group 3 (n = 29) (%)Group 4 (n = 49) (%)Total (n = 119) (%)P
    Sex.02a
        Male7 (46.7)13 (50)20 (69)39 (80)79 (66.4)
        Female8 (53.3)13 (50)9 (31)10 (20)40 (33.6)
    Age at diagnosis (yr)b8.7 (3.1)6.2 (4.6)6.1 (3.8)8.1 (3.2)7.3 (3.8)<.0001a
    Histology.02a
        LCA1 (6.7)9 (34.6)13 (44.8)7 (14.3)30 (25.2)
        Classic14 (93.3)8 (30.8)15 (51.7)39 (79.6)76 (63.9)
        Desmoplastic0 (0)9 (34.6)1 (3.5)3 (6.1)13 (10.9)
    • Note:—LCA indicates large-cell anaplastic.

    • ↵a Statistically significant.

    • ↵b Mean with SDs.

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    Table 2:

    Locations of primary tumors with respect to molecular groups

    SubgroupFourth VentricleCPA (n = 6) (%)Paraventricular (n = 12) (%)Cerebellar Periphery (n = 19) (%)
    No. (n = 119) (%)Midline-Vermis (n = 40) (%)4th-uniCPA (n = 25) (%)4th-biCPA (n = 17) (%)Subtotal (n = 81) (%)
    WNT15 (12.6)5 (12.5)3 (12)1 (5.9)8 (9.9)3 (50)3 (25)0 (0)
    SHH26 (21.8)3 (7.5)0 (0)0 (0)3 (3.7)3 (50)2 (16.7)18 (94.7)
    Group 329 (24.4)11 (27.5)8 (32)6 (35.3)25 (30.9)0 (0)4 (33.3)0 (0)
    Group 449 (41.2)21 (52.5)14 (56)10 (58.8)45 (55.5)0 (0)3 (25)1 (5.3)
    • Note:—4th-uniCPA indicates fourth ventricle with unilateral extension along the lateral recess toward the CPA; 4th-biCPA, fourth ventricle with bilateral extension along bilateral recesses toward the CPAs.

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    Table 3:

    Imaging features of initial metastases with respect to their molecular group assignmenta

    MRI SignalsInitial Metastasis (n = 25)
    WNT (n = 0) (%)SHH (n = 3) (%)Group 3 (n = 8) (%)Group 4 (n = 14) (%)P Valueb
    Ependymal C−/D+0 (0)0 (0)(0)6 (42.8).01c
    3rd V.I.R, C−/D+d0 (0)(0)(0)4 (28.6).02c
    Ependymal C+/D+0 (0)0 (0)3 (37.5)0 (0).02c
    Leptomeningeal C−/D+0 (0)0 (0)0 (0)3 (21.4).05
    Leptomeningeal C+/D+0 (0)3 (100)5 (62.5)5 (35.7).17
    Leptomeningeal C+/D−0 (0)0 (0)0 (0)4 (28.6).02c
    • Note:—C− indicates no contrast enhancing; D+, diffusion-restricting; D−, no diffusion-restricting; C+, contrast-enhancing; 3rd V.I.R, third ventricle infundibular recess.

    • ↵a The sum of metastases in some locations is higher than the total number of patients in the group because each case could have multiple metastases in >1 compartment.

    • ↵b P value determined using the Fisher exact test.

    • ↵c Statistically significant.

    • ↵d Subgroup of patients with mismatching ependymal metastasis in the third ventricle infundibular recess.

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American Journal of Neuroradiology: 39 (5)
American Journal of Neuroradiology
Vol. 39, Issue 5
1 May 2018
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MRI Characteristics of Primary Tumors and Metastatic Lesions in Molecular Subgroups of Pediatric Medulloblastoma: A Single-Center Study
D. Mata-Mbemba, M. Zapotocky, S. Laughlin, M.D. Taylor, V. Ramaswamy, C. Raybaud
American Journal of Neuroradiology May 2018, 39 (5) 949-955; DOI: 10.3174/ajnr.A5578

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MRI Characteristics of Primary Tumors and Metastatic Lesions in Molecular Subgroups of Pediatric Medulloblastoma: A Single-Center Study
D. Mata-Mbemba, M. Zapotocky, S. Laughlin, M.D. Taylor, V. Ramaswamy, C. Raybaud
American Journal of Neuroradiology May 2018, 39 (5) 949-955; DOI: 10.3174/ajnr.A5578
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