Imaging Features of Juvenile Xanthogranuloma of the Pediatric Head and Neck

Ginat, Daniel Thomas; Vargas, Sara O.; Silvera, V. Michelle; Volk, Mark S.; Degar, Barbara; Robson, Caroline D.

doi:10.3174/ajnr.a4644

Cited by 28 publications

(25 citation statements)

References 27 publications

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“…In accordance with other reports [30, 31] diagnostic work-up with ultrasound showed a well-defined, homogeneous, hypoechoic lesion without demonstrable blood flow in the dermis (Patient 1) or viscera (Patient 2) in both patients [30, 31].…”

Section: Discussionsupporting

confidence: 91%

“…In the literature, enhancement is described as a reliable feature of JXG lesions [32]. The typical imaging ranges from iso- to hyperintense on T1 and iso- to hypointense on T2 [31, 33]. MRI findings in our Patient 2 showed the big thoracic tumor conglomerate on T1 and on T2 slightly hyperintense to muscle, furthermore multiple nodular lesions in the liver, hyperintense in TIRM and T2 and hypointense in T1-weighted sequences.…”

Section: Discussionmentioning

confidence: 72%

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The various clinical spectra of juvenile xanthogranuloma: imaging for two case reports and review of the literature

et al. 2019

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Background Juvenile xanthogranuloma (JXG) belongs to the heterogeneous group of non-Langerhans cell histiocytosis and is caused by an accumulation and proliferation of macrophages. In the majority of cases JXG is a disorder of early childhood presenting during the first 2 years of life. The typical presentation is a solitary reddish or yellowish skin papule or nodule with spontaneous regression and no need for treatment. Case presentation Two infants with an atypical presentation of JXG, one with multiple blueberry muffin rash-like skin lesions and the other with severe multi-systemic involvement, are reported. Diagnosis was established by skin biopsy including histological work-up and immunostaining, where markers for macrophages (CD68 and CD163) exhibited significant reactivity. Conclusion JXG is the most common of the non-Langerhans cell histiocytosis. The typical presentation is a solitary skin lesion. The purpose of this report is to familiarize paediatricians with an unusual variant of this entity in order to facilitate early diagnosis and raise awareness for possible visceral complications and associated medical conditions.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 72%

The various clinical spectra of juvenile xanthogranuloma: imaging for two case reports and review of the literature

et al. 2019

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“…In published reports, 9 , 10 the appearance of intracranial JXG lesions on MRI varies. Lesions are usually round and are hypointense, isointense or slightly hyperintense on T1-weighted or T2-weighted images.…”

Section: Discussionmentioning

confidence: 99%

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

Jin

Deng

et al. 2020

J Int Med Res

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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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“…W niektórych przypadkach obserwuje się zmiany pozaskórne (multifocal JXG). Mogą one wystąpić w obrębie tkanki podskórnej, gałki ocznej, ślinianki, a także każdego innego narządu wewnętrznego (wątroba, nerki, śledziona, trzustka, płuca, OUN, kości) [9][10][11][12][13][14][15]. Zmiany narządowe mogą wpływać na przeżycie pacjentów, prowadząc do niewydolności narządowej, ucisku otaczających struktur lub krwotoków [16,17].…”

Section: Opis Przypadkuunclassified

“…In some cases, the presence of extracutaneous lesions (multifocal JXG) is observed. Lesions can occur within the subcutaneous tissue, eyeball, salivary gland, and practically any internal organ (liver, kidneys, spleen, pancreas, lungs, CNS, bones) [9][10][11][12][13][14][15]. Organ changes may affect the survival of patients, leading to organ failure, compression of surrounding structures or hemorrhages [16,17].…”

Section: Opis Przypadkumentioning

confidence: 99%

Multifocal juvenile xanthogranuloma

Jarliński¹,

Węgłowska²

2020

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Imaging Features of Juvenile Xanthogranuloma of the Pediatric Head and Neck

Abstract: BACKGROUND AND PURPOSE:Juvenile xanthogranuloma is a non-Langerhans cell histiocytosis primarily affecting children. The purpose of this study was to characterize the imaging features of histologically confirmed pediatric head and neck juvenile xanthogranuloma.

Cited by 28 publications

References 27 publications

The various clinical spectra of juvenile xanthogranuloma: imaging for two case reports and review of the literature

The various clinical spectra of juvenile xanthogranuloma: imaging for two case reports and review of the literature

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

Multifocal juvenile xanthogranuloma

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