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Longitudinal Assessment of Enhancing Foci of Abnormal Signal Intensity in Neurofibromatosis Type1


Hainc, N; Wagner, M W; Laughlin, S; Rutka, J; Hawkins, C; Blaser, S; Ertl-Wagner, B B (2021). Longitudinal Assessment of Enhancing Foci of Abnormal Signal Intensity in Neurofibromatosis Type1. American Journal of Neuroradiology, 42(4):766-773.

Abstract

BACKGROUND AND PURPOSE

Patients with neurofibromatosis 1 are at increased risk of developing brain tumors, and differentiation from contrast-enhancing foci of abnormal signal intensity can be challenging. We aimed to longitudinally characterize rare, enhancing foci of abnormal signal intensity based on location and demographics.

MATERIALS AND METHODS

A total of 109 MR imaging datasets from 19 consecutive patients (7 male; mean age, 8.6 years; range, 2.3-16.8 years) with neurofibromatosis 1 and a total of 23 contrast-enhancing parenchymal lesions initially classified as foci of abnormal signal intensity were included. The mean follow-up period was 6.5 years (range, 1-13.8 years). Enhancing foci of abnormal signal intensity were followed up with respect to presence, location, and volume. Linear regression analysis was performed.

RESULTS

Location, mean peak volume, and decrease in enhancing volume over time of the 23 lesions were as follows: 10 splenium of the corpus callosum (295 mm$^{3}$, 5 decreasing, 3 completely resolving, 2 surgical intervention for change in imaging appearance later confirmed to be gangliocytoma and astrocytoma WHO II), 1 body of the corpus callosum (44 mm$^{3}$, decreasing), 2 frontal lobe white matter (32 mm$^{3}$, 1 completely resolving), 3 globus pallidus (50 mm$^{3}$, all completely resolving), 6 cerebellum (206 mm$^{3}$, 3 decreasing, 1 completely resolving), and 1 midbrain (34 mm$^{3}$). On average, splenium lesions began to decrease in size at 12.2 years, posterior fossa lesions at 17.1 years, and other locations at 9.4 years of age.

CONCLUSIONS

Albeit very rare, contrast-enhancing lesions in patients with neurofibromatosis 1 may regress over time. Follow-up MR imaging aids in ascertaining regression. The development of atypical features should prompt further evaluation for underlying tumors.

Abstract

BACKGROUND AND PURPOSE

Patients with neurofibromatosis 1 are at increased risk of developing brain tumors, and differentiation from contrast-enhancing foci of abnormal signal intensity can be challenging. We aimed to longitudinally characterize rare, enhancing foci of abnormal signal intensity based on location and demographics.

MATERIALS AND METHODS

A total of 109 MR imaging datasets from 19 consecutive patients (7 male; mean age, 8.6 years; range, 2.3-16.8 years) with neurofibromatosis 1 and a total of 23 contrast-enhancing parenchymal lesions initially classified as foci of abnormal signal intensity were included. The mean follow-up period was 6.5 years (range, 1-13.8 years). Enhancing foci of abnormal signal intensity were followed up with respect to presence, location, and volume. Linear regression analysis was performed.

RESULTS

Location, mean peak volume, and decrease in enhancing volume over time of the 23 lesions were as follows: 10 splenium of the corpus callosum (295 mm$^{3}$, 5 decreasing, 3 completely resolving, 2 surgical intervention for change in imaging appearance later confirmed to be gangliocytoma and astrocytoma WHO II), 1 body of the corpus callosum (44 mm$^{3}$, decreasing), 2 frontal lobe white matter (32 mm$^{3}$, 1 completely resolving), 3 globus pallidus (50 mm$^{3}$, all completely resolving), 6 cerebellum (206 mm$^{3}$, 3 decreasing, 1 completely resolving), and 1 midbrain (34 mm$^{3}$). On average, splenium lesions began to decrease in size at 12.2 years, posterior fossa lesions at 17.1 years, and other locations at 9.4 years of age.

CONCLUSIONS

Albeit very rare, contrast-enhancing lesions in patients with neurofibromatosis 1 may regress over time. Follow-up MR imaging aids in ascertaining regression. The development of atypical features should prompt further evaluation for underlying tumors.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Neuroradiology
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:1 April 2021
Deposited On:03 Mar 2021 15:23
Last Modified:13 Apr 2021 01:12
Publisher:American Society of Neuroradiology
ISSN:0195-6108
OA Status:Closed
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:https://doi.org/10.3174/ajnr.A6974
PubMed ID:33541905

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