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

Research ArticleSpine Imaging and Spine Image-Guided Interventions

Spinal and Paraspinal Plexiform Neurofibromas in Patients with Neurofibromatosis Type 1: A Novel Scoring System for Radiological-Clinical Correlation

M. Mauda-Havakuk, B. Shofty, S. Ben-Shachar, L. Ben-Sira, S. Constantini and F. Bokstein
American Journal of Neuroradiology October 2017, 38 (10) 1869-1875; DOI: https://doi.org/10.3174/ajnr.A5338
M. Mauda-Havakuk
aFrom the Gilbert Israeli NF Center (M.M.-H., B.S., S.B.-S., L.B.-S., S.C., F.B.)
bPediatric Radiology Unit (M.M.-H., L.B.-S.)
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  • ORCID record for M. Mauda-Havakuk
B. Shofty
aFrom the Gilbert Israeli NF Center (M.M.-H., B.S., S.B.-S., L.B.-S., S.C., F.B.)
cPediatric Neurosurgery (B.S., S.C.)
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S. Ben-Shachar
aFrom the Gilbert Israeli NF Center (M.M.-H., B.S., S.B.-S., L.B.-S., S.C., F.B.)
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L. Ben-Sira
aFrom the Gilbert Israeli NF Center (M.M.-H., B.S., S.B.-S., L.B.-S., S.C., F.B.)
bPediatric Radiology Unit (M.M.-H., L.B.-S.)
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S. Constantini
aFrom the Gilbert Israeli NF Center (M.M.-H., B.S., S.B.-S., L.B.-S., S.C., F.B.)
cPediatric Neurosurgery (B.S., S.C.)
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F. Bokstein
aFrom the Gilbert Israeli NF Center (M.M.-H., B.S., S.B.-S., L.B.-S., S.C., F.B.)
dNeuro-Oncology Service (F.B.), Dana Children's Hospital, Tel-Aviv Medical Center, and Tel-Aviv University, Tel-Aviv, Israel.
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    Fig 1.

    Complex spinal manifestation in a patient with NF1. Spinal MR imaging study of a 36-year-old asymptomatic patient with NF1. Note that the tumors involve all the neuroforamina in the cervical and lumbar segments (A and C). Axial T1 with gadolinium demonstrates massive paraspinal involvement of the thoracic region (B). Sagittal T2 with fat saturation of the lumbar region demonstrates multiple tumors in the neuroforamina and in the lumbar spine (C).

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    Fig 2.

    Schematic representation of our classification of spinal involvement in NF1 (A). A red circle represents tumors within the neuroforamina (group 1). Blue circles represent bilateral tumors within the neuroforamina with sac compression (kissing neurofibromas, group 2). The green ellipse represents paraspinal involvement (group 3). The purple ellipse represents an intradural lesion (group 4). A patient with this imaging result would have received a score of 6 for cervical foraminal, 2 for thoracic, 2 for lumbar foraminal, 1 for thoracic paraspinal, 1 for intradural thoracic, and 2 for 2 pairs of lumbar kissing tumors. The spinal cord schema image has been released as part of an open knowledge project by Cancer Research UK and attributed to Cancer Research UK/Wikimedia Commons. B, T2 with fat saturation coronal MR imaging study of the lumbar region of a patient with NF1. In the yellow rectangle, we see an example of 2 pairs of bilateral tumors approximating each other at the same level to <1–2 mm, with significant compression of the thecal sac (kissing PN). Note the large paraspinal component of the tumors (group 1).

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    Fig 3.

    Spinal tumor distribution in asymptomatic and symptomatic patients. Error bars represent the standard error of the mean.

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    Fig 4.

    Spinal tumor distribution in patients with and without pain. Error bars represent the standard error of the mean.

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    Fig 5.

    Spinal tumor distribution in patients with neurologic deficits and in neurologically intact patients. Patients with neurologic deficits had more paraspinal tumors in the cervical region (group 1) and more kissing NF (group 3) in the cervical and the lumbar regions. Error bars represent the standard error of the mean.

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    Fig 6.

    Radiologic risk scores for different patient groups. Symptomatic patients (with neurologic deficits and/or pain) had higher scores compared with asymptomatic patients (A). Patients showing neurologic deterioration had higher scores compared with patients with stable conditions (B).

Tables

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  • Detailed tumor distribution for the entire cohort (n = 34)

    Group 1: NeuroforaminalaGroup 2: Kissing NeurofibromasaGroup 3: ParaspinalbGroup 4: Intraduralb
    Cervical7.3 ± 6 (0–14)0.8 ± 1.9 (0–6)10 (29%)3 (8%)
    Thoracic4.9 ±7.5 (0–24)013 (38%)3 (8%)
    Lumbo-sacral8.8 ± 7.7 (0–20)1.6 ± 3.5 (0–16)15 (44%)5 (14%)
    • ↵a The average number ± SD of involved levels in each spinal region for each tumor type for the entire cohort (n = 34). Numbers in parentheses represent the range of affected levels.

    • ↵b Number of patients (%). Note that for intradural and paraspinal tumors, the number represents the total number of patients, regardless of how many levels are affected.

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American Journal of Neuroradiology: 38 (10)
American Journal of Neuroradiology
Vol. 38, Issue 10
1 Oct 2017
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Cite this article
M. Mauda-Havakuk, B. Shofty, S. Ben-Shachar, L. Ben-Sira, S. Constantini, F. Bokstein
Spinal and Paraspinal Plexiform Neurofibromas in Patients with Neurofibromatosis Type 1: A Novel Scoring System for Radiological-Clinical Correlation
American Journal of Neuroradiology Oct 2017, 38 (10) 1869-1875; DOI: 10.3174/ajnr.A5338

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Spinal and Paraspinal Plexiform Neurofibromas in Patients with Neurofibromatosis Type 1: A Novel Scoring System for Radiological-Clinical Correlation
M. Mauda-Havakuk, B. Shofty, S. Ben-Shachar, L. Ben-Sira, S. Constantini, F. Bokstein
American Journal of Neuroradiology Oct 2017, 38 (10) 1869-1875; DOI: 10.3174/ajnr.A5338
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