Desmoplastic Fibroma of the Skull;A Case Report, Review of the Literature, and Therapeutic Implications

ABSTRACT

Desmoplastic fibroma(DF) is a rare neoplasm of the bone, and is histologically benign but locally aggressive disease. A total of nine cases of DF involving skull have been reported in the literature, and among these eight are females. In this report, the clinical findings and histopathology of a case with DF of the skull in a male patient is presented with a review of the literature with an emphasis on treatment modalities. A 21-year-old man presented with headache. CT scan revealed a solitary and lytic skull lesion without brain invasion. DF was confirmed by histological evaluation. On immunohistochemical staining of the tumor was negative for estrogen or progesterone receptors. After total resection of tumor with wide surgical margin, there was no recurrence during the 35 months of follow-up period. Although longer follow up period maybe needed, treatment of this type of tumor with complete resection of tumor tissue along with a wide margin may provide long disease-free state compare to the high recurrence rates in DF of other sites.