Aggressive intracranial fibromatosis: case report

Fibromatosis is a locally aggressive, proliferative fibroblastic lesion affecting musculoaponeurotic structures, most often in the limbs and trunk. Intracranial fibromatosis is extremely rare and requires aggressive treatment to prevent recurrence. We present the case of a 48 year old woman with aggressive skull base fibromatosis. The lesion extended through the sphenoid sinus, into both pterygoid recesses, destroying the right lateral wall of the sphenoid sinus and invading the cavernous sinus. There was also involvement of the floor of the sella, the clivus, the right petrous temporal bone and the right mastoid. The patient underwent partial resection of the lesion via an extended trans-sphenoidal approach. Postoperative MRI showed residual tissue. A review of the literature shows that intracranial fibromatosis usually appears in the first or second decade. Complete resection is often impossible because of its widely infiltrative nature. Radiotherapy and chemotherapy are often required to improve local control of the lesion.