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Research ArticlePediatric Neuroimaging

Neuroimaging in Pediatric Patients with Juvenile Xanthogranuloma of the CNS

B.L. Serrallach, S.F. Kralik, B.H. Tran, T.A.G.M. Huisman, R.P. Patel, C.E. Allen, K.L. McClain, N. Gulati, C.Q. Dillard-Ilboudo, M.J. Hicks, C.A. Mohila and N.K. Desai
American Journal of Neuroradiology October 2022, DOI: https://doi.org/10.3174/ajnr.A7683
B.L. Serrallach
aFrom the Edward B. Singleton Department of Radiology (B.L.S., S.F.K., B.H.T., T.A.G.M.H., R.P.P., N.K.D.)
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S.F. Kralik
aFrom the Edward B. Singleton Department of Radiology (B.L.S., S.F.K., B.H.T., T.A.G.M.H., R.P.P., N.K.D.)
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B.H. Tran
aFrom the Edward B. Singleton Department of Radiology (B.L.S., S.F.K., B.H.T., T.A.G.M.H., R.P.P., N.K.D.)
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T.A.G.M. Huisman
aFrom the Edward B. Singleton Department of Radiology (B.L.S., S.F.K., B.H.T., T.A.G.M.H., R.P.P., N.K.D.)
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R.P. Patel
aFrom the Edward B. Singleton Department of Radiology (B.L.S., S.F.K., B.H.T., T.A.G.M.H., R.P.P., N.K.D.)
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C.E. Allen
bDepartment of Pediatrics (C.E.A., K.L.M., N.G., C.Q.D.-I.), Section of Hematology-Oncology
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K.L. McClain
bDepartment of Pediatrics (C.E.A., K.L.M., N.G., C.Q.D.-I.), Section of Hematology-Oncology
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N. Gulati
bDepartment of Pediatrics (C.E.A., K.L.M., N.G., C.Q.D.-I.), Section of Hematology-Oncology
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C.Q. Dillard-Ilboudo
bDepartment of Pediatrics (C.E.A., K.L.M., N.G., C.Q.D.-I.), Section of Hematology-Oncology
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M.J. Hicks
cDepartment of Pathology and Immunology (M.J.H., C.A.M.), Texas Children’s Hospital and Baylor College of Medicine, Houston, Texas
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C.A. Mohila
cDepartment of Pathology and Immunology (M.J.H., C.A.M.), Texas Children’s Hospital and Baylor College of Medicine, Houston, Texas
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N.K. Desai
aFrom the Edward B. Singleton Department of Radiology (B.L.S., S.F.K., B.H.T., T.A.G.M.H., R.P.P., N.K.D.)
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  • FIG 1.
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    FIG 1.

    Brain images of a 6-year-old girl with pathology-proven unifocal JXG who presented with increased frequency of headaches, occasionally accompanied by vomiting. Brain MR imaging with an axial T2-weighted image (A), axial T1-weighted image (B), axial DWI (C), axial ADC map (D), axial T1+CE-weighted image (E), and axial noncontrast CT (F) shows a large, round, unifocal mass lesion located in the right frontal lobe (arrows in A–F) with central necrosis (arrowhead in E), perilesional edema, and right-to-left midline shift (arrowhead in A). Compared with cortical GM, the solid non-necrotic peripheral component of the lesion is T2-isointense (A) and T1-isointense (B) and shows contrast enhancement (E) and restricted diffusion (C and D). The axial CT image (F) demonstrates the hyperdense (solid portion), large mass lesion.

  • FIG 2.
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    FIG 2.

    Brain MR images of a 3-year-old boy with pathology-proven multifocal JXG and new-onset strabismus. Axial T2-weighted (A–C), axial T1-weighted (D–F), and axial T1+CE-weighted (G–I) MR imaging sequences show exemplary 3 round-to-oval lesions located in the right (arrows in A, D, and G) and left paracentral (arrows in B, E, and H) regions and the pons (arrows in C, F, and I), respectively. Compared with cortical GM, the lesions are T2-iso- to hyperintense (A–C) and T1-hyperintense (D–F) and show homogeneous contrast enhancement (G–I) and mild perilesional edema.

  • FIG 3.
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    FIG 3.

    MR images obtained in a 10-year-old boy with pathology-proven multifocal JXG with leptomeningeal brain and spine involvement. The patient presented with progressive right-ear pain and erythema and decreased hearing and right-sided facial nerve palsy along with headaches. Axial (A, B, and D) and coronal T1+CE-weighted (C) MR images of the brain. E, Sagittal T1+CE-weighted fat-suppressed MR image of the spine. For example, 2 centrally necrotic, peripherally contrast-enhancing lesions are noted within both cerebral hemispheres (arrows in A). In addition, nodular contrast enhancement involving the leptomeninges of the right temporal lobe (arrow in B) and contrast enhancement within both internal auditory canals (arrows in C and D) are shown. Sagittal T1+CE-weighted images of the spine depict increased contrast enhancement of the leptomeninges (“sugar coating”), partially with nodular components, most obvious at the C7 and T8 level (arrows in E).

Tables

  • Figures
  • Imaging features of CNS JXGa

    No.T1-WeightedT2-WeightedT1+CEDWI/ADC
    4/12↔↔4 Marked contrast enhancement4 Diffusion restriction
    3/12↔↔ - ↑ or ↑3 Marked contrast enhancement1 Diffusion restriction, 2 no diffusion restriction
    2/12↑↑2 Marked contrast enhancement1 Diffusion restriction, 1 NA
    2/12↑↔ or ↔ - ↑1 Marked contrast enhancement/1 with lesions with marked and lesions without contrast enhancement1 No diffusion restriction, 1 NA
    1/12↓ - ↔↓ - ↔1 Marked contrast enhancement1 Diffusion restriction
    • Note:—↔ indicates isointense; ↑, hyperintense; ↔ - ↑, iso- to hyperintense; ↓ - ↔, hypo- to isointense; NA, not applicable.

    • ↵a Cases were included only when both T1WI and T2WI were available.

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Cite this article
B.L. Serrallach, S.F. Kralik, B.H. Tran, T.A.G.M. Huisman, R.P. Patel, C.E. Allen, K.L. McClain, N. Gulati, C.Q. Dillard-Ilboudo, M.J. Hicks, C.A. Mohila, N.K. Desai
Neuroimaging in Pediatric Patients with Juvenile Xanthogranuloma of the CNS
American Journal of Neuroradiology Oct 2022, DOI: 10.3174/ajnr.A7683

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Neuroimaging in Pediatric Patients with Juvenile Xanthogranuloma of the CNS
B.L. Serrallach, S.F. Kralik, B.H. Tran, T.A.G.M. Huisman, R.P. Patel, C.E. Allen, K.L. McClain, N. Gulati, C.Q. Dillard-Ilboudo, M.J. Hicks, C.A. Mohila, N.K. Desai
American Journal of Neuroradiology Oct 2022, DOI: 10.3174/ajnr.A7683
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