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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Research ArticlePediatric Neuroimaging

Longitudinal Assessment of Enhancing Foci of Abnormal Signal Intensity in Neurofibromatosis Type 1

N. Hainc, M.W. Wagner, S. Laughlin, J. Rutka, C. Hawkins, S. Blaser and B.B. Ertl-Wagner
American Journal of Neuroradiology April 2021, 42 (4) 766-773; DOI: https://doi.org/10.3174/ajnr.A6974
N. Hainc
aFrom the Department of Diagnostic Imaging (N.H., M.W.W., S.L., S.B., B.B.E.-W.), Division of Neuroradiology
dDepartment of Neuroradiology (N.H.), Clinical Neuroscience Center, University Hospital Zurich, University of Zurich, Switzerland
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  • ORCID record for N. Hainc
M.W. Wagner
aFrom the Department of Diagnostic Imaging (N.H., M.W.W., S.L., S.B., B.B.E.-W.), Division of Neuroradiology
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S. Laughlin
aFrom the Department of Diagnostic Imaging (N.H., M.W.W., S.L., S.B., B.B.E.-W.), Division of Neuroradiology
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J. Rutka
bDepartment of Surgery, Division of Neurosurgery (J.R.)
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C. Hawkins
cDepartment of Paediatric Laboratory Medicine (C.H.), The Hospital for Sick Children and Department of Laboratory Medicine & Pathobiology, University of Toronto, Canada
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S. Blaser
aFrom the Department of Diagnostic Imaging (N.H., M.W.W., S.L., S.B., B.B.E.-W.), Division of Neuroradiology
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B.B. Ertl-Wagner
aFrom the Department of Diagnostic Imaging (N.H., M.W.W., S.L., S.B., B.B.E.-W.), Division of Neuroradiology
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  • FIG 1.
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    FIG 1.

    Diagram showing the number of patients with NF-1 included in the study.

  • FIG 2.
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    FIG 2.

    Combined box-and-whisker plot of the temporal evolution of FASI in the brain stem and cerebellum (BS and CB) group (left box-and-whisker plot in the doublet), basal ganglia and thalamus (BG and T) group (right box-and-whisker plot in the doublet), and enhancing FASI (EF, total of all patients, continuous curved black line), grouped in 2-year patient-age intervals, beginning at 2–3.9 years. Contrast-enhancing FASI appear later and peak later (11 lesions, 10- to 11.9-year-old age group) compared with nonenhancing FASI (BS/CB peak in 6- to 7.9-year-old age group and BG/T peak in 8- to 9.9-year-old age group).

  • FIG 3.
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    FIG 3.

    Horizontal plot of all volumetrically assessed contrast-enhancing FASI. White bars represent imaging studies where no contrast enhancement was noted. The white-to-black (left to right) gradient represents increasing enhancing volume; the black-to-white (left-to-right) gradient represents decreasing enhancing volume. The numbers to the left and right of the bars indicate the enhancing FASI volume on first and last MR imaging. The numbers on the far right indicate the largest measured enhancing volume for the respective lesion; all values are in cubic millimeters. All 8 lesions within the splenium are seen to either decrease in size or completely resolve before age 18 years (not including the 2 lesions later proved to be tumors). Both globus pallidus lesions completely resolved before age 11 years. Only 3 of 6 posterior fossa (cerebellum and midbrain) lesions were seen to decrease in size on final MR imaging. Body of corpus callosum (1 lesion) and frontal lobe lesions (2 lesions) did not show any conceivable trends. The 2 contrast-enhancing FASI not available for viewing in the PACS are not included in this figure.

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    FIG 4.

    Linear regression analysis of splenium lesions (A); posterior fossa lesions, including the cerebellum and midbrain (B); and other lesions, including the globus pallidus, body of the corpus callosum, and frontal lesions (C). Enhancing FASI lesion volumes are expressed as ratios of interval change divided by maximum lesion volume. The x-intercept represents the transition point at which lesions begin to decrease in size. Splenium lesions began decreasing in size at 12.2 years, posterior fossa at 17.1 years, and other lesions at 9.4 years of age.

  • FIG 5.
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    FIG 5.

    Typical (A–D) and atypical (E–H) evolutions of enhancing FASI. A–D demonstrate, a lesion in the splenium or right forceps major increasing in size to age 13 years (B) and decreasing thereafter with near complete resolution of contrast enhancement on final imaging (D). E–H represent an atypical contrast enhancement pattern in a histologically proved WHO II astrocytoma. This lesion initially demonstrated evolution identical to FASI, increasing in size up to age 15 years (F) before beginning to regress (G). At this point, however, the small right periventricular enhancing aspect again began increasing in size at age 17 years (H). At this point, the lesion was resected because this was considered to be atypical for an enhancing FASI. Mass effect was noted in both lesions.

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    Table 1:

    Patient age group data for enhancing FASI (total) and nonenhancing FASI according to subgroup

    Age Group (years)2–3.94–5.96–7.98–9.910–11.912–13.914–15.916–17.9
    Participants5781517161314
    Enhancing FASI
     Total011711997
     Splenium of CC25442
     Body of CC11
     Frontal lobe111
     Globus pallidus1111a1a
     Cerebellum2442 + 1a3
     Midbrain011
    Nonenhancing FASI BG and T
     Total1829398386542726
     Mean3.64.14.95.35.13.42.11.9
     Range0–71–81–100–111–111–100–40–3
    Nonenhancing FASI BS and CB
     Total658589121141815162
     Mean6.56.15.644.12.322.2
     Range0–132–120–140–110–90–71–30–8
    Nonenhancing FASI splenium CC
     Total6751127352333
     Mean1.210.60.71.62.21.82.4
     Range0–30–30–30–20–50–71–31–6
    Nonenhancing FASI remainder CC
     Total22113302
     Mean0.80.30.10.10.20.200.1
     Range0–20–10–10–10–10–100–1
    • Note:—BG indicates basal ganglia; BS, brain stem; CB, cerebellum; CC, corpus callosum; T, thalamus.

    • ↵a Lesions with images not available for volumetric analysis.

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American Journal of Neuroradiology: 42 (4)
American Journal of Neuroradiology
Vol. 42, Issue 4
1 Apr 2021
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N. Hainc, M.W. Wagner, S. Laughlin, J. Rutka, C. Hawkins, S. Blaser, B.B. Ertl-Wagner
Longitudinal Assessment of Enhancing Foci of Abnormal Signal Intensity in Neurofibromatosis Type 1
American Journal of Neuroradiology Apr 2021, 42 (4) 766-773; DOI: 10.3174/ajnr.A6974

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Longitudinal Assessment of Enhancing Foci of Abnormal Signal Intensity in Neurofibromatosis Type 1
N. Hainc, M.W. Wagner, S. Laughlin, J. Rutka, C. Hawkins, S. Blaser, B.B. Ertl-Wagner
American Journal of Neuroradiology Apr 2021, 42 (4) 766-773; DOI: 10.3174/ajnr.A6974
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