Multiple Intracranial Xanthogranulomas : Case Report

A 58-year-old female was admitted to our hospital with nausea, vomiting, and gait disturbance of 1 year duration. Postcontrast computed tomographic scans demonstrated enhanced lesions in the left cerebellopontine angle (CPA), the retrosellar region, the right parasellar region, and the left parietooccipital convexity. The left parieto-occipital tumor was totally removed in the first operation and the left CPA tumor was partially removed in the second. The histological diagnosis of both tumors was xanthogranuloma. She also had cutaneous lesions (subcutaneous nodules without tenderness) and an ureteral stenosis possibly due to the retroperitoneal involvement. A skin biopsy demonstrated infiltration of xanthoma cells and foamy cells in the dermis. A gallium scintigram demonstrated an abnormal uptake in the thoracic cavity, liver, and bones. From these findings, systemic Weber-Christian disease was diagnosed. Another unique aspect was that the serum IgE levels were increased during postoperative hospitalization. This suggests that abnormal immunological conditions are related to this disease and that intracranial xanthogranulomas are a manifestation of systemic Weber-Christian disease.