Juvenile xanthogranuloma: unusual intraoral finding

Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis (non-LCH) affecting normolipemic infants and children most frequently in the first year of life, often showing spontaneous regression within 3 to 6 years. Classic JXG is characterized by a yellowish asymptomatic papule or nodule, often located in the skin of the head, neck and upper trunk. Oral JXG has been reported, but is rare. Histologically, JXG is composed mainly of an infiltrate of macrophages with a variable degree of lipidization (foamy macrophages), and (most of the time) scattered Touton-type giant cells. Because of the rarity of oral lesions and possible variations in the clinical and histological presentation, the correct diagnosis can be challenging, requiring a careful clinical and histopathological evaluation with adjuvant immunohistochemical studies. Our review of the English-language literature disclosed 33 cases of oral JXG, including this case report. The purpose of this study is to present a new case of this uncommon entity as well as to review and discuss its main clinicopathologic features and immunohistochemical findings.

show abstract

“…Three cases presented skin lesions (Ossoff et al, Sangüeza et al and Collins et al), and none visceral involvement.…”

Section: Discussionmentioning

confidence: 95%

Oral juvenile xanthogranuloma in a child: Clinical, histological and immunohistochemical profile of a rare entity

et al. 2018

View full text Add to dashboard Cite

show abstract

“…Extracutaneous lesions of the head and neck have been reported in numerous anatomic spaces: subcutaneous fat (Figs. 2A, B, 3A-E, 4A-H), paranasal sinuses, nasal cavity, trachea, 11 subglottis, oral cavity and lips, [12][13][14] orbit, tympanic membrane, external auditory canal, parotid gland, temporal bone, intracranial compartment, and cervical spine. 5 Here, we report the largest series of head and neck JXG at various locations.…”

Section: Discussionmentioning

confidence: 99%

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

Chalard,

Nguyen,

Morel

et al. 2024

Journal of Pediatric Hematology/Oncology

View full text Add to dashboard Cite

Background: Juvenile Xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis, occurring mainly in infancy. With an extracutaneous lesion, its diagnosis is difficult, because of a wide clinical spectrum. Here we demonstrate and characterize imaging features of 11 patients with JXG of the head and neck in various locations. Material and Methods: We recorded clinical data and reviewed all imaging studies of 11 patients with JXG of the head and neck. Ultrasonography (US) alone was performed in 1 patient; MRI alone in 6 patients; US and MRI in 1 patient; and US, CT, and MRI in 3 patients. We evaluated the following characteristics in all studies: location and number of lesions, echogenicity and vascularization on US, density on CT, signal intensity on T1- and T2-weighted images, ADC and enhancement on MRI, and tumor boundaries and bone involvement. Results: Lesions were well-defined in 9 cases, and bone erosion was present in 2. On US, lesions were hypoechoic or hyperechoic and with or without vascularization. On CT, lesions were hyper-dense, with no calcification. On MRI, lesions were mildly hyper-intense or iso-intense on T1-weighted images in 8 of 9 patients, hypo-intense on T2-weighted images in 7 of 10, low ADC in 7 of 9, and enhancement in 7 of 7. Conclusions: The diagnosis of extra cutaneous JXG may be proposed, with the following suggestive criteria: age < 1 year, well-defined lesion, mild hyper-intensity on T1-weighted images, hypo-intensity on T2-weighted images, low ADC, enhancement, and possible adjacent bone involvement.

show abstract

“…Cuando se presenta afección única se conoce como XGJ solitario. Se ha descrito que afecta más a los lactantes varones (1.5 veces) que a niñas, pero al llegar a la edad adulta no hay distinciones por sexo (3,4).…”

Section: Discussionunclassified

Xantogranuloma juvenil : reporte de caso.

et al. 2016

View full text Add to dashboard Cite

Introducción: el xantogranuloma juvenil (XGJ) se encuentra dentro de las histiocitosis de células de Langerhans. Es descrita como una enfermedad benigna, caracterizada por la presencia de neoformaciones pápulo-nodulares en la primera infancia (rara en los adultos) y tiene un carácter autolimitado y autoinvolutivo. Raramente afecta órganos internos; histológicamente se caracteriza por inclusiones lipídicas en histiocitos sin alteraciones metabólicas de base. El objetivo es presentar el caso de una preescolar femenina que consultó el servicio de Dermatología Pediátrica por lesión en región axilar.Caso clínico: paciente femenina de dos años y diez meses de edad con antecedentes de nacimiento a término sin complicaciones y esquema de vacunación completo para su edad. Requirió hospitalización a los cinco meses de edad por bronquiolitis aguda durante cinco días, sin necesidad de ingresar a unidad de cuidados intensivos, ni ventilación mecánica. La paciente presentaba antecedente paterno de rinitis alérgica.Conclusión: las lesiones dermatológicas sugestivas de XGJ deben tratarse si causan síntomas o para establecer su diagnóstico. Usualmente son benignas, aunque existen condiciones especiales a considerar. Rev.cienc.biomed. 2016;7(2):306-309.

show abstract

Juvenile xanthogranuloma: unusual intraoral finding

Cited by 9 publications

References 8 publications

Oral juvenile xanthogranuloma in a child: Clinical, histological and immunohistochemical profile of a rare entity

Oral juvenile xanthogranuloma in a child: Clinical, histological and immunohistochemical profile of a rare entity

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

Xantogranuloma juvenil : reporte de caso.

Contact Info

Product

Resources

About