Fibroxanthoma arising from the cranial dura mater

A primary xanthomatous tumor is very rare in the central nervous system (CNS). Here we report the case of a fibroxanthoma arising from the dura mater of the cerebrum that demonstrated no systemic disease or metabolic abnormalities. A 19-month-old, otherwise healthy boy was found to have an enlarged head. Magnetic resonance imaging demonstrated a left occipital dural mass lesion and an enlarged left cerebral hemisphere with ipsilateral ventricular enlargement. Subtotal removal of the tumor was performed through the left parieto-occipital craniotomy. The tumor was composed of a central fibrous portion, a peripheral xanthomatous area, and a boundary. The peripheral area of the tumor showed abundant uniform xanthomatous cells with a thin fibrous stroma and the mass was diagnosed as fibroxanthoma involving the dura. This may represent a distinct category of tumor, which is different from the previously reported cases of fibrous xanthoma and fibrous histiocytoma. Intracranial xanthomatous tumors may be heterogeneous in their origin and histological features. However, further studies are needed to elucidate their clinical features, biological behavior, and optimal treatment strategies.