Imaging features of neonatal systemic juvenile xanthogranuloma: a case report and review of the literature

Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytic disorder in children. This report describes the case of a 28-day-old boy that presented with multiple subcutaneous nodular lesions on the trunk and extremities, and multiple red nodular lesions on the scrotum. Magnetic...

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Main Authors: Siping He, Ke Jin, Xicheng Deng, Zhengzhen Zhou, Robert C McKinstry, Yong Wang
Format: Article
Language:English
Published: SAGE Publishing 2020-09-01
Series:Journal of International Medical Research
Online Access:https://doi.org/10.1177/0300060520956416
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author Siping He
Ke Jin
Xicheng Deng
Zhengzhen Zhou
Robert C McKinstry
Yong Wang
author_facet Siping He
Ke Jin
Xicheng Deng
Zhengzhen Zhou
Robert C McKinstry
Yong Wang
author_sort Siping He
collection DOAJ
description Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytic disorder in children. This report describes the case of a 28-day-old boy that presented with multiple subcutaneous nodular lesions on the trunk and extremities, and multiple red nodular lesions on the scrotum. Magnetic resonance imaging (MRI) of the brain showed a well-demarcated extra-axial dura-based mass that appeared isointense or slightly hyperintense on T1-weighted images, hypointense on T2-weighted images and had intense enhancement on gadolinium-enhanced T1-weighted images. Computed tomography (CT) or MRI scans of the chest and abdomen revealed multiple scattered nodular or patchy lesions of varying sizes in the lungs, liver and left kidney. Histological analysis of a subcutaneous mass suggested JXG. The patient was diagnosed with neonatal systemic JXG with involvement of the central nervous system, lungs, liver, kidneys, subcutaneous soft tissue and skin. CT and MRI after 3 months of treatment with methylprednisolone sodium succinate demonstrated that the lesions were obviously diminished. This report discusses the imaging findings in this current case of multi-organ JXG and reviews the imaging literature on this condition to improve awareness of the lesions in order to help radiologists establish an accurate differential diagnosis when confronted with similar cases.
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spelling doaj.art-3de337e305ce457eb85f52e315008cd02022-12-21T19:51:04ZengSAGE PublishingJournal of International Medical Research1473-23002020-09-014810.1177/0300060520956416Imaging features of neonatal systemic juvenile xanthogranuloma: a case report and review of the literatureSiping HeKe JinXicheng DengZhengzhen ZhouRobert C McKinstryYong WangJuvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytic disorder in children. This report describes the case of a 28-day-old boy that presented with multiple subcutaneous nodular lesions on the trunk and extremities, and multiple red nodular lesions on the scrotum. Magnetic resonance imaging (MRI) of the brain showed a well-demarcated extra-axial dura-based mass that appeared isointense or slightly hyperintense on T1-weighted images, hypointense on T2-weighted images and had intense enhancement on gadolinium-enhanced T1-weighted images. Computed tomography (CT) or MRI scans of the chest and abdomen revealed multiple scattered nodular or patchy lesions of varying sizes in the lungs, liver and left kidney. Histological analysis of a subcutaneous mass suggested JXG. The patient was diagnosed with neonatal systemic JXG with involvement of the central nervous system, lungs, liver, kidneys, subcutaneous soft tissue and skin. CT and MRI after 3 months of treatment with methylprednisolone sodium succinate demonstrated that the lesions were obviously diminished. This report discusses the imaging findings in this current case of multi-organ JXG and reviews the imaging literature on this condition to improve awareness of the lesions in order to help radiologists establish an accurate differential diagnosis when confronted with similar cases.https://doi.org/10.1177/0300060520956416
spellingShingle Siping He
Ke Jin
Xicheng Deng
Zhengzhen Zhou
Robert C McKinstry
Yong Wang
Imaging features of neonatal systemic juvenile xanthogranuloma: a case report and review of the literature
Journal of International Medical Research
title Imaging features of neonatal systemic juvenile xanthogranuloma: a case report and review of the literature
title_full Imaging features of neonatal systemic juvenile xanthogranuloma: a case report and review of the literature
title_fullStr Imaging features of neonatal systemic juvenile xanthogranuloma: a case report and review of the literature
title_full_unstemmed Imaging features of neonatal systemic juvenile xanthogranuloma: a case report and review of the literature
title_short Imaging features of neonatal systemic juvenile xanthogranuloma: a case report and review of the literature
title_sort imaging features of neonatal systemic juvenile xanthogranuloma a case report and review of the literature
url https://doi.org/10.1177/0300060520956416
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