Bilateral intracranial fibrous xanthoma

Abstract

We present a case of fibrous xanthoma in a 6-year-old boy who showed disturbance of consciousness and signs of increased intracranial pressure. A computed tomographic (CT) scan showed bitemporal enhanced masses and hydrocephalus. The patient was operated on in two stages. The tumors were located intradurally and extracerebrally and were firmly attached to the dura mater at the base of the middle fossa. Glial fibrillary acidic protein stain was negative, and histologic examination suggested a diagnosis of bilateral fibrous xanthoma. Postoperative CT scan showed residual tumors and an enlarged choroid plexus adjacent to it. A CT scan at 12 years of age showed no enhanced tumor, but disclosed bitemporal high-density areas and diffuse brain atrophy; the choroid plexus reverted to normal size. The patient showed mental and physical retardation, perhaps owing to the compression of the hypothalamus during early childhood. This would appear to be the first known case of bilateral intracranial fibrous xanthoma.