Diagnostic and management difficulties in a case of multiple intracranial juvenile xanthogranuloma

Juvenile xanthogranuloma (JXG) preferentially occurs in childhood, and its characteristics have been thought to be benign and with slow growth. JXG is classified as an inflammatory disease, which forms multiple lesions in the patients' head, neck, and other organs and typically skin. JXG is rare, and few case reports have been published in the past, particularly with multiple intracranial lesions, which as in our case, is an extremely rare finding.

Patient is a 4-year-old boy who presented with polydypsia and polyuria for 1 year. He had been followed up only under l-deamino-8-D-arginine vasopressin until he started vomiting and his level of consciousness deteriorated. Then, magnetic resonance imaging (MRI) revealed multiple intracranial lesions. He underwent biopsy via small craniotomy, and pathology was confirmed as juvenile xanthogranuloma. The patient received chemotherapy, in complete compliance with JLSG-02 protocol.

JXG is characteristic with homogenous enhancement with contrast matter and, most importantly, high intensity on diffusion-weighted image (DWI). The DWI was high when the degree of diffusion of water is restricted, as seen in inflammation and, additionally, the change of intensity after administration of steroid would reflect its anti-inflammatory nature. However, though the steroid therapy made high-intensity lesions decrease signal intensity, the size and the number of lesions did not change at all. As we expected, after we stopped steroid administration to the patient, his consciousness deteriorated and we found again the very-high-intensity lesions. We consider the intensity on DWI to reflect progression of the lesions.