Intracranial chondromyxoid fibroma. Report of a case and review of the literature.

Chondromyxoid fibroma is an unusual benign tumor of cartilaginous derivation. We describe a rare example of chondromyxoid fibroma of the frontal-sphenoid junction with orbital infiltration in a 35-year-old Hispanic woman who presented with frontal headaches. Gross total excision was performed. The excised mass was composed of neoplastic cells with chondrocytic features within a myxoid matrix. Bony infiltration was present without infiltration of dura mater or brain tissue. The lack of mitotic activity, low cell density, lack of nuclear pleomorphism, and a fused lobular architectural pattern indicated that the lesion was a chondromyxoid fibroma. The lack of hyaline cartilage helped differentiate the lesion from enchondroma. Our case demonstrates the uncommon occurrence of intracranial chondromyxoid fibroma with orbital infiltration. When faced with an intracranial chondrocytic tumor, it is important to distinguish this neoplasm from enchondroma and chondrosarcoma.