Multiple intracranial juvenile xanthogranulomas

The authors report on an 11-year-old boy in whom proptosis of the eye cause d by a benign intraosseous xanthofibroma of the left orbital wall became cl inically apparent at the age of 4 years. Two years later he developed bilat eral papilledema, at which time computerized tomography and magnetic resona nce studies revealed multiple enhancing intracranial lesions. The largest m ass was located in the left middle fossa; other lesions were located at the tentorium cerebelli, in both lateral ventricles, near the superior sagitta l sinus, and extracranially near the left jugular vein. The mass in the lef t middle fossa was resected and diagnosed as juvenile xanthogranuloma (JXG) . Thirty months later, the patient again became symptomatic, exhibiting beh avioral abnormalities and a decrease in mental powers. At that time, the tw o remaining lesions in both lateral ventricles had grown enough to cause tr apping of the temporal horns and raised intracranial pressure. These lesion s were successively resected and histopathologically confirmed to be JXGs. However, resection of the second intraventricular lesion was complicated by postoperative bilateral amaurosis, presumably caused by postdecompression optic neuropathy. According to a review of the literature, fewer than 20 pa tients with JXG involving the central nervous system have been reported. Th e patient described in this report is the first in whom multiple intracrani al JXGs developed in the absence of cutaneous manifestations. Although JXGs are biologically benign lesions, the treatment of patients with multifocal and/or progressive intracranial manifestations is problematic.