Bilateral intracranial fibrous xanthoma

Area of Science:

• Neuropathology
• Pediatric Neurosurgery

Background:

• Fibrous xanthoma is a rare tumor, with intracranial presentation being exceptionally uncommon, particularly in pediatric cases.

Observation:

• A 6-year-old boy presented with altered consciousness and signs of increased intracranial pressure, indicative of a serious neurological condition.
• Computed tomography (CT) scans revealed bitemporal enhanced masses and hydrocephalus, suggesting significant intracranial pathology.
• Surgical intervention identified intradural and extracerebral tumors attached to the dura mater at the base of the middle fossa.

Findings:

• Histological examination, with negative glial fibrillary acidic protein staining, supported a diagnosis of bilateral fibrous xanthoma.
• Postoperative imaging showed residual tumors and an enlarged choroid plexus, with long-term follow-up revealing no enhanced tumor but persistent bitemporal high-density areas and diffuse brain atrophy.
• The patient experienced mental and physical retardation, potentially linked to hypothalamic compression during early development.

Implications:

• This case represents the first documented instance of bilateral intracranial fibrous xanthoma, expanding the known spectrum of this rare tumor.
• The findings underscore the importance of considering rare diagnoses in pediatric neurological presentations and the potential for long-term neurodevelopmental consequences.
• Further research into the etiology and optimal management of intracranial fibrous xanthoma is warranted.