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Research ArticleAdult Brain

Multinodular and Vacuolating Posterior Fossa Lesions of Unknown Significance

A. Lecler, J. Bailleux, B. Carsin, H. Adle-Biassette, S. Baloglu, C. Bogey, F. Bonneville, E. Calvier, P.-O. Comby, J.-P. Cottier, F. Cotton, R. Deschamps, C. Diard-Detoeuf, F. Ducray, L. Duron, C. Drissi, M. Elmaleh, J. Farras, J.A. Garcia, E. Gerardin, S. Grand, D.C. Jianu, S. Kremer, N. Magne, M. Mejdoubi, A. Moulignier, M. Ollivier, S. Nagi, M. Rodallec, J.-C. Sadik, N. Shor, T. Tourdias, C. Vandendries, V. Broquet and J. Savatovsky for the ENIGMA Investigation Group (EuropeaN Interdisciplinary Group for MVNT Analysis)
American Journal of Neuroradiology October 2019, 40 (10) 1689-1694; DOI: https://doi.org/10.3174/ajnr.A6223
A. Lecler
aFrom the Departments of Neuroradiology (A.L., J.B., L.D., J.-C.S., C.V., V.B., J.S.)
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J. Bailleux
aFrom the Departments of Neuroradiology (A.L., J.B., L.D., J.-C.S., C.V., V.B., J.S.)
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B. Carsin
cDepartment of Radiology (B.C., J.S.), Centre Hospitalier Régional Universitaire de Rennes, Rennes, France
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H. Adle-Biassette
dDepartment of Pathology (H.A.-B.), Lariboisière Hospital, Paris Diderot, Paris-Cité-Sorbonne University, Paris, France
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S. Baloglu
eDepartment of Radiology (S.B., S.K.), University Hospital of Strasbourg, Strasbourg, France
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  • ORCID record for S. Baloglu
C. Bogey
fDepartment of Neuroradiology (C.B.), Centre Hospitalier Universitaire Limoges, Limoges, France
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  • ORCID record for C. Bogey
F. Bonneville
gDepartment of Neuroradiology (F.B.), Hôpital Pierre-Paul-Riquet, Centre Hospitalier Universitaire Purpan, Toulouse, France
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E. Calvier
hNeurology Department (E.C., J.A.G.), Hôpital René et Guillaume-Laënnec, Centre Hospitalier Universitaire de Nantes, Saint-Herblain, France
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P.-O. Comby
iDepartment of Vascular and Interventional Radiology (P.-O.C.), Image-Guided Therapy Center, François-Mitterrand University Hospital, Dijon Cedex, France
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J.-P. Cottier
jDepartment of Radiology (J.-P.C.), Centre Hospitalier Régional Universitaire de Tours, Tours, France
kBrain and Imaging Laboratory (J.-P.C.), UMR U930, National Institute for Health and Medical Research, François-Rabelais University, Tours, France
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F. Cotton
lService de Radiologie (F.C.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, University Claude Bernard Lyon 1, Lyon, France
mCentre de Recherche en Acquisition et Traitement de l'Image pour la Santé (CREATIS) (F.C.), National Institute for Health and Medical Research U1044/Centre National de la Recherche Scientifique (CNRS) Unité Mixte de Recherche (UMR) 5220, Lyon, France
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R. Deschamps
bNeurology (R.D., A.M.), Fondation Ophtalmologique A. Rothschild, Paris, France
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C. Diard-Detoeuf
nDepartment of Neurology (C.D.-D.), CH Sainte-Périne, Paris, France
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F. Ducray
oDepartment of Neuro-Oncology (F.D.), Lyon French Reference Center of Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, Bron Cedex, France
pSynatac Team (F.D.), NeuroMyoGene Institut, National Institute for Health and Medical Research U1217/Centre National de la Recherche Scientifique (CNRS) Unité Mixte de Recherche (UMR) 5310, Lyon, France
qUniversity Claude Bernard Lyon 1 (F.D.), Lyon, France
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L. Duron
aFrom the Departments of Neuroradiology (A.L., J.B., L.D., J.-C.S., C.V., V.B., J.S.)
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C. Drissi
rInstitut National de Neurologie (C.D., S.N.), Service de Neuroradiologie, Université de Tunis El Manar, Faculté de Médecine de Tunis, Tunis, Tunisia
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M. Elmaleh
sPediatric Radiology Department (M.E.), Robert Debré Hospital, Paris, France
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J. Farras
tJordi Radiologia C/de la Roda (J.F.), Andorra la Vella, Andorra
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J.A. Garcia
hNeurology Department (E.C., J.A.G.), Hôpital René et Guillaume-Laënnec, Centre Hospitalier Universitaire de Nantes, Saint-Herblain, France
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E. Gerardin
uDepartment of Neuroradiology and MRI (E.G., N.M.), Rouen University Hospital, Rouen, France
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S. Grand
vNeuroradiologie Diagnostique et Interventionnelle et IRM Nord (S.G.), Centre Hospitalier et Universitaire de Alpes Grenoble, Grenoble, France
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D.C. Jianu
wDepartment of Neurology (D.C.J.), Victor Babes University of Medicine and Pharmacy, Timisoara, Romania
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S. Kremer
eDepartment of Radiology (S.B., S.K.), University Hospital of Strasbourg, Strasbourg, France
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N. Magne
uDepartment of Neuroradiology and MRI (E.G., N.M.), Rouen University Hospital, Rouen, France
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M. Mejdoubi
xDepartment of Neuroradiology (M.M.), University Hospital of Martinique, Fort-de-France, Martinique, France
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A. Moulignier
bNeurology (R.D., A.M.), Fondation Ophtalmologique A. Rothschild, Paris, France
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M. Ollivier
yGroupe Hospitalier Pellegrin (M.O.), Bordeaux, France
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S. Nagi
rInstitut National de Neurologie (C.D., S.N.), Service de Neuroradiologie, Université de Tunis El Manar, Faculté de Médecine de Tunis, Tunis, Tunisia
zClinique les Berges du Lac (S.N.), les Berges du Lac, Tunis, Tunisia
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M. Rodallec
aaCentre d'Imagerie Centre Cardiologique du Nord (M.R.), CCN, Saint-Denis, France
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J.-C. Sadik
aFrom the Departments of Neuroradiology (A.L., J.B., L.D., J.-C.S., C.V., V.B., J.S.)
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N. Shor
abDepartment of Neuroradiology (N.S.), Pitié-Salpêtrière Hospital, Paris, France
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T. Tourdias
acService de Neuroimagerie Diagnostique et Thérapeutique (T.T.), Centre Hospitalier Universitaire de Bordeaux et National Institute for Health and Medical Research U1215, Université de Bordeaux, Bordeaux, France
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C. Vandendries
aFrom the Departments of Neuroradiology (A.L., J.B., L.D., J.-C.S., C.V., V.B., J.S.)
adCentre d'Imagerie Médicale Paris 15ème (C.V.), RMX, Paris, France
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V. Broquet
aeDepartment of Neuroradiology (V.B.), Centre Hospitalier Universitaire Lille, Lille, France
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J. Savatovsky
aFrom the Departments of Neuroradiology (A.L., J.B., L.D., J.-C.S., C.V., V.B., J.S.)
cDepartment of Radiology (B.C., J.S.), Centre Hospitalier Régional Universitaire de Rennes, Rennes, France
afImagerie Paris 13 (J.S.), Paris, France.
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    Fig 1.

    A 38-year-old man presenting with headache. 3D-T2-FLAIR reformatted in the axial (A), coronal (B), and sagittal (C) planes shows a high-signal intensity lesion (white arrow) of the posterior part of the left cerebellar peduncle, consisting of a coalescence of small nodules, highly suggestive of MV-PLUS. The lesion is hypointense on axial T1WI (D) and does not enhance on postcontrast T1WI (E). SWI (F) shows no blooming or intratumoral susceptibility signal. High-resolution T2WI (G) shows hypointensity in the center of hyperintense nodules (black arrows), consistent with a central dot sign. Note the small mass effect and distortion of the lateral margin of the fourth ventricle.

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    Fig 2.

    A 31-year-old woman presenting with headache. 3D-T2-FLAIR reformatted in the sagittal (A) and axial (B) planes shows a high signal intensity multinodular lesion (arrow) of the upper vermis, highly suggestive of an MV-PLUS. Almost all clustered nodules are hypointense on 3D-T1WI reformatted in the sagittal (C) and axial (D) planes and do not enhance on postcontrast 3D-T1WI reformatted in the sagittal (E) and axial (F) planes. One anterior nodule (arrowhead) shows a substantially higher T2-FLAIR and lower T1 signal intensity than all the others, with a marked enhancement after contrast injection. Note the T2-FLAIR hypointense central dot sign does not enhance on postcontrast T1WI.

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    Table 1:

    Patient characteristics and clinical data

    No. of Patients (n = 11)Percentage (%)
    Sex
        Male545
        Female655
    Age (mean) (yr)37 ± 13
    Clinical symptoms prompting initial imaging
        Headache436
        Meningioma screening218
        Bilateral upper arm paresthesia19
        Hearing loss19
        Tinnitus19
        Dizziness19
    Aneurysm screening19
    • View popup
    Table 2:

    MR imaging characteristics of multinodular and vacuolating posterior fossa lesions of unknown significance (MV-PLUS) at diagnosis and during follow-up

    No. of PatientsPercentage (%)
    Location
        Center of the lesion
            Cerebellum
                Vermis7/1164
                Left cerebellar hemisphere1/119
                Right cerebellar hemisphere2/1118
                Left cerebellar peduncle1/119
                Right cerebellar peduncle0/110
            Brain stem0/110
        All areas involved
            Cerebellum
                Vermis7/1164
                Left cerebellar hemisphere3/1127
                Right cerebellar hemisphere8/1173
                Left cerebellar peduncle1/119
                Right cerebellar peduncle0/110
            Brain stem0/110
    Overall size (median) (IQR) (mm)27 (24)
    Size of the nodules (median) (IQR) (mm)4 (5)
    DWI
        Restriction0/110
    T2* or SWI
        Presence of ITSS0/110
        Blooming0/110
    PWI
        rCBV (median) (IQR)0.62 (0.06)
        rCBF (median) (IQR)0.61 (0.06)
    MRS
        TE = 35 ms
            Choline/creatine (median) (IQR)0.9 (0.2)
            Choline/N-acetyl aspartate (median) (IQR)0.9 (0.1)
            N-acetyl aspartate/creatine (median) (IQR)1 (0.2)
        TE = 135 ms
            Choline/creatine (median) (IQR)1.2 (0)
            Choline/N-acetyl aspartate (median) (IQR)1 (0)
            N-acetyl aspartate/creatine (median) (IQR)1.2 (0)
    Type of margins
        Sharp11/11100
        Blurred0/110
    Mass effect
        Yes4/1136
        No7/1164
    Associated imaging abnormalities
        None7/1164
        Small-vessel disease3/1127
        Colloid cyst1/119
        Cholesteatoma1/119
    • Note:—ITSS indicates intratumoral susceptibility signal; rCBV, relative CBV; rCBF, relative CBF.

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A. Lecler, J. Bailleux, B. Carsin, H. Adle-Biassette, S. Baloglu, C. Bogey, F. Bonneville, E. Calvier, P.-O. Comby, J.-P. Cottier, F. Cotton, R. Deschamps, C. Diard-Detoeuf, F. Ducray, L. Duron, C. Drissi, M. Elmaleh, J. Farras, J.A. Garcia, E. Gerardin, S. Grand, D.C. Jianu, S. Kremer, N. Magne, M. Mejdoubi, A. Moulignier, M. Ollivier, S. Nagi, M. Rodallec, J.-C. Sadik, N. Shor, T. Tourdias, C. Vandendries, V. Broquet, J. Savatovsky
Multinodular and Vacuolating Posterior Fossa Lesions of Unknown Significance
American Journal of Neuroradiology Oct 2019, 40 (10) 1689-1694; DOI: 10.3174/ajnr.A6223

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Multinodular and Vacuolating Posterior Fossa Lesions of Unknown Significance
A. Lecler, J. Bailleux, B. Carsin, H. Adle-Biassette, S. Baloglu, C. Bogey, F. Bonneville, E. Calvier, P.-O. Comby, J.-P. Cottier, F. Cotton, R. Deschamps, C. Diard-Detoeuf, F. Ducray, L. Duron, C. Drissi, M. Elmaleh, J. Farras, J.A. Garcia, E. Gerardin, S. Grand, D.C. Jianu, S. Kremer, N. Magne, M. Mejdoubi, A. Moulignier, M. Ollivier, S. Nagi, M. Rodallec, J.-C. Sadik, N. Shor, T. Tourdias, C. Vandendries, V. Broquet, J. Savatovsky
American Journal of Neuroradiology Oct 2019, 40 (10) 1689-1694; DOI: 10.3174/ajnr.A6223
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American Society of Neuroradiology

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Print ISSN: 0195-6108 Online ISSN: 1936-959X

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