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

Abstract

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.

Congenital Tumors—Magnetic Resonance Imaging Findings with Focus on Rare Tumors

“…There are only a few reports of systemic disease but none of them presented the involvement pattern similar to that in our patient. He et al described a patient with multiple subcutaneous nodular lesions on the trunk and extremities, multiple bilateral peripheral pulmonary nodules, and also abdominal changes, but the hypodense lesions in the liver and left kidney were completely different from our patient [49,50]. similar to that in our patient.…”

“…similar to that in our patient. He et al described a patient with multiple subcutane nodular lesions on the trunk and extremities, multiple bilateral peripheral pulmon nodules, and also abdominal changes, but the hypodense lesions in the liver and left ney were completely different from our patient [49,50]. Finally in our material there are two cases of an unusual condition that was asses on MRI as tumors-blue rubber bleb nevus syndrome (BRBNS, Bean syndrome).…”

Juvenile Xanthogranuloma of the Head and Neck: Imaging Findings in 11 Cases

“… 69 Conversely, in a series of 14 cases, Serralach et al found that JXG lesions were iso-intense on T 1 -weighted images and iso- or hyperintense on T 2 -weighted images. 70 On diffusion-weighted sequences, a low ADC is generally reported, 7 , 38 , 66 , 68 and Chiba et al considered the intensity on diffusion-weighted imaging to reflect the progression of the lesions. Perilesional edema is absent, mild 38 , 41 , 43 and or significant.…”

“…8 However, some radiologic characteristics could be identified. On MRI, lesion enhancement is common 7,38,47,48,[64][65][66] and is the most frequent imaging finding by Wang et al, with necrotic, cystic, and hemorrhagic changes less common. 48 Moreover, Su et al reported increased perfusion in the JXG, associated with enhancement.…”

“…48 Moreover, Su et al reported increased perfusion in the JXG, associated with enhancement. 65 Iso-to hypersignals on T 1 -weighted images and isosignals to hyposignals on T 2 -weighted images have been noted, 7,30,47,[64][65][66][67][68] which Chen et al attributed to the fatty content and attenuated cellularity, respectively, in 27 patients. Nevertheless, such an iso/hyposignal on T 2weighted images is not pathognomonic, and is also found in fibroblastic and myofibroblastic tumors.…”