Disseminated juvenile xanthogranuloma occurring after treatment of Langerhans cell histiocytosis: a case report.

CASE PRESENTATION

An 11-year-old boy presented with a complaint of a painful temporal mass. Brain magnetic resonance imaging (MRI) showed a 3-cm-sized, homogeneously enhancing mass in the greater wing of the left sphenoid bone, which was diagnosed as Langerhans cell histiocytosis (LCH). Chemotherapy with vincristine and prednisolone was performed for 1 year. After 1 year and 11 months off treatment, he developed symptoms such as polydipsia and polyuria. Brain MRI showed thickening of the pituitary stalk with enhancement, suggestive of LCH involvement, and no recurrence in the sphenoid bone. After 4 years and 4 months off treatment, he developed multiple, subcutaneous, asymptomatic, and yellowish variable-sized papules on his face, posterior neck, and back, which were pathologically diagnosed as juvenile xanthogranuloma (JXG). Brain MRI revealed multifocal enhancing skull lesions in the left parietal, right frontal, and left occipital bones, which were also diagnosed as JXG. After 5 years and 8 months off treatment, the number of variable-sized skin lesions was increased without changes in the lesions in the skull and pituitary stalk.

CONCLUSION

We report a case of disseminated JXG occurring after treatment of LCH. These clinical co-presentations suggested a close relationship between their pathogenesis.