Intracranial disseminated Juvenile xanthogranuloma with a space-occupying lesion in the chest: A case report

Abstract Background Juvenile xanthogranuloma (JXG) is a type of non-Langerhans cell histiocytosis (non-LCH) that primarily affects infants and young children. It mostly occurs in the skin, while the brain is rarely affected. Multiple intracranial lesions, as found in this case, are even more scarce. Case We are reporting the case of a 9-year-old boy who presented with an imbalanced gait, ataxia, nystagmus, and right facial droop. Magnetic resonance imaging (MRI) revealed multiple intracranial lesions, while chest contrast-enhanced computed tomography (CT) showed irregular soft tissue density of the lesion, with uneven density, poorly defined border, and mild heterogeneous enhancement. Histopathologic examination of the resected intracranial lesion reported disseminated JXG. Subsequently, the patient received treatment of methylprednisolone and interferon-α. The three-month follow-up CT showed that the lesion within the chest was slightly reduced. Conclusion This case report aims to highlight the radiological features, diagnosis, and treatment of intracranial JXG and to provide some guidance for clinical management. Given the rarity of such cases and the lack of relevant studies, further research regarding to diagnosis and treatment is needed.