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Aggressive fibromatosis (or desmoid tumor) refers to a histologically benign but locally aggressive lesion arising from musculoaponeurotic structures in various anatomic sites. Extra-abdominal desmoids represent about one third of all desmoid tumors; of these only about 11 to 15% arise in the head and neck. Desmoid tumors arising in the infratemporal fossa are exceedingly rare; to our knowledge only one such tumor has been reported in the literature. We present a desmoid tumor arising in the infratemporal fossa with intracranial extension in a twenty-seven year old male and review the literature on this rare condition.
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