2017
DOI: 10.1177/1203475417690718
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A Rare Case of Solitary Giant Congenital Juvenile Xanthogranuloma: A Case Report

Abstract: Juvenile xanthogranuloma (JXG) is the most frequent form of non-Langerhans cell histiocytosis. We present a case of giant congenital JXG in a 7-week-old boy, who had a firm and incompressible lesion, measuring 3 × 4 cm in diameter, on his right flank. The clinical appearance of the lesion and the ultrasound results suggested a vascular tumor, such as a hemangioma. Histology confirmed a JXG, although there was an absence of Touton cells, which are usually pathognomonic of JXG. In light of these findings, it wou… Show more

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Cited by 7 publications
(12 citation statements)
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“…To our knowledge, 51 cases of giant JXG have been reported in the English literature. 8,10-50 We analyzed all cases of giant JXG for various characteristics. In our analysis, we also compared giant JXG and classic JXG (Table 1).…”
Section: Discussionmentioning
confidence: 99%
“…To our knowledge, 51 cases of giant JXG have been reported in the English literature. 8,10-50 We analyzed all cases of giant JXG for various characteristics. In our analysis, we also compared giant JXG and classic JXG (Table 1).…”
Section: Discussionmentioning
confidence: 99%
“…Lesions are typically small, with only a handful of reported cases larger than 20 mm—referred to as giant JXGs . There is no evidence that these behave differently from non‐giant JXGs, although presentation is atypical, and lesions are often described clinically as isolated vascular masses with a pale or yellowish center …”
Section: Discussionmentioning
confidence: 99%
“…The characteristic Touton cell contains a “wreath” of nuclei surrounded by foamy cytoplasm. Immunohistochemistry in JXG is typically positive for CD68 and Factor XIIIa and negative for S‐100, helping to distinguish from LCH, which is usually positive for CD1a, Langerin, and S‐100 …”
Section: Discussionmentioning
confidence: 99%
“…Die Riesenvariante der kongenitalen JXG ist sehr selten; es gibt in der Literatur nur sehr wenige Berichte darüber [5][6][7][8][9][10]. Sie wird als eine Läsion definiert, die grösser als 2 cm ist und ähnliche klinische und histopathologische Merkmale wie das klassische JXG aufweist, mit Ausnahme des bereits erwähnten höheren Risikos einer extrakutanen Beteiligung.…”
Section: Diskussionunclassified