Juvenile Xanthogranuloma in a Child: A Case Report

Background Juvenile xanthogranuloma (JXG) is a rare non-Langerhans cell histiocytic disorder that predominantly affects infants and young children. It most commonly presents as a solitary cutaneous lesion, while extracutaneous involvement is considerably less frequent. Case presentation: We report the case of a 7 -year-old girl who presented with The patient presented with headache, weakness in the left upper and lower extremities, inability to walk independently, nausea, vomiting, dysphagia, shortness of breath, and generalized weakness for approximately 5 months. Clinical examination revealed Clinical examination revealed left-sided hemiparesis involving both the upper and lower extremities, impaired independent ambulation, and signs of dysphagia. The patient was generally weak, with reduced muscle strength in the left extremities. Magnetic resonance imaging demonstrated a mass lesion was identified in the right cerebral hemisphere. The patient underwent a right-sided pterional craniotomy with surgical resection of the right temporal lobe tumor. Histopathological and immunohistochemical examination demonstrated diffuse expression of CD68 and CD163 in macrophages and giant cells, with focal expression of Factor XIIIa and S100. Immunohistochemical staining for CD1a was negative. The morphological and immunophenotypic findings were consistent with juvenile xanthogranuloma (JXG).No data regarding cerebrospinal fluid (CSF) analysis obtained by lumbar puncture were available. During the course of treatment, the patient received CapmaXen (capmatinib) for 2 days and Kotellic (cobimetinib) for 12 days. Conclusion JXG Juvenile xanthogranuloma should be considered in the differential diagnosis of intracranial mass lesions in pediatric patients, particularly in cases with atypical neurological manifestations and no characteristic cutaneous involvement. Histopathological and immunohistochemical evaluation is essential for establishing an accurate diagnosis, especially in patients with isolated or atypical intracranial disease.