Solitary juvenile xanthogranuloma mimicking intracranial tumor in children

Juvenile xanthogranuloma (JXG) is primarily a benign cutaneous disorder of non-Langerhans hystiocytic proliferation. Systemic involvement occurs in 4% of patients; isolated central nervous system (CNS) lesions are rare. We report solitary CNS-JXG lesions in two patients. A 3.5-year-old boy with a parietal-occipital lesion underwent total resection with no surgical morbidity and no recurrence at 16-month follow-up. A 3.5-year-old girl underwent subtotal resection of a tumor extending from the left Meckel’s cave and invading the cavernous sinus and left orbit with extensive cranial nerve involvement. Tumor regrowth with leptomeningeal spread at 9-month and 12-month follow-up was managed with steroids and chemotherapy (vinblastine and later cladribine). We present our experience and review the literature pertaining to rare reports of solitary CNS-JXG.