Mononuclear variant of juvenile xanthogranuloma in the oral cavity of an adult patient

Nonlipidized juvenile xanthogranuloma: An unusual variant with a potential diagnostic pitfall

“…The most affected sites in the oral cavity are the tongue, palate, gingiva, and lip [16,17]. Four out of 31 oral cases of JXG with non-classic microscopical features, showing an epithelioid-shaped histiocytic population with ''benign appareance'' and described as ''early JXG'' or ''mononuclear variant of JXG'', have been reported [13,14]. The case described here is the sixth JXG affecting the lip, but the first with mononuclear/monomorphic and nonlipidized cells and presenting ''malignant appareance''.…”

“…To date, about 28 NJXG cases affecting extra-oral sites have been described in the literature [2,[4][5][6][7][8][9][10][11][12]. Only 4 cases of non-classic JXG involving the mouth, described as ''early JXG'' or ''mononuclear variant of JXG'', have been reported [13,14] and none of them showed mitotic activity nor it was considered the possibility of malignancy. We report an unusual case of NJXG in a 6-year-old Brazilian boy which showed a clinically ulcerated nodule on the mucosa of the lower lip, histologically mimicking a malignant neoplasm.…”

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

“…However, there are other less frequent clinicopathologic variants of JXG such as nonlipidized and mononuclear variants, which could even be confused with a malignant mesenchymal neoplasm. The mononuclear JXG, presents vacuolar foamy histocytes in the absence of giant cells, and because of this feature, it may be misdiagnosed as LCH, melanoma or balloon cell nevus . In these cases, a careful clinical evaluation, histopathological analysis and immunohistochemistry are needed to make a correct diagnosis.…”

Coexistence of Classic and a Mononuclear Variant of Juvenile Xanthogranuloma in an Adult Patient

“…Juvenile xanthogranuloma (JXG) is histologically characterized by dense mononuclear cells with foamy histiocytes and Touton giant cells in a background of lymphocytes and eosinophils 1 2 3 . An unusual variant of non-lipidized JXG has been described in approximately 28 cases, in which Touton giant cells and foam cells are absent or very scant.…”

“…An unusual variant of non-lipidized JXG has been described in approximately 28 cases, in which Touton giant cells and foam cells are absent or very scant. These cases are described as non-lipidized JXG, early JXG, or a mononuclear JXG variant 1 3 4 5 . This variant is typically found in infants or children younger than three years and presents most commonly as a solitary lesion 1 4 .…”