DESMOPLASTIC FIBROMA OF THE SKULL - CASE-REPORT AND REVIEW OF THE LITERATURE

Background and purpose. - We present a rare case of desmoplastic fibro ma of the skull. Desmoplastic fibroma is a distinctive and rare neopla sm of bone that histologically and biologically mimics desmoid soft ti ssue tumor. Only 6 cases have been reported in the skull and none of t hese was diagnosed by MRI. Clinical presentation. - A 64 year-old woma n, operated on in June 1991 for left mastectomy due to a ductal adenoc arcinoma and again in October 1994 for a left frontal metastatic adeno carcinoma, was admitted in our Department of Neurosurgery because a co ntrol cerebral MRI had detected a diploic lesion, isointense on T1-wei ghted images and hyperintense on T2, with moderate enhancement, locali zed in a right parietal site, Neurological examination was negative. T he lesion was surgically removed and a cranioplasty was performed. His tological diagnosis was desmoplastic fibroma. Twelve months after trea tment she has no neurological symptoms or signs of cerebral lesions (M RI) or systemic metastasis (total body CT). Conclusion. - In the liter ature the number of desmoplastic fibroma is too small and the follow-u p period too short to permit any conclusions regarding the aggressiven ess of the tumor.