P Celli1, L Cervoni, G Trillò. 1. Department of Neurological Sciences II, La Sapienza University, Rome.
Abstract
BACKGROUND AND PURPOSE: We present a rare case of desmoplastic fibroma of the skull. Desmoplastic fibroma is a distinctive and rare neoplasm of bone that histologically and biologically mimics desmoid soft tissue tumor. Only 6 cases have been reported in the skull and none of these was diagnosed by MRI. CLINICAL PRESENTATION: A 64 year-old woman, operated on in June 1991 for left mastectomy due to a ductal adenocarcinoma and again in October 1994 for a left frontal metastatic adenocarcinoma, was admitted in our Department of Neurosurgery because a control cerebral MRI had detected a diploic lesion, isointense on T1-weighted images and hyperintense on T2, with moderate enhancement, localized in a right parietal site. Neurological examination was negative. The lesion was surgically removed and a cranioplasty was performed. Histological diagnosis was desmoplastic fibroma. Twelve months after treatment she has no neurological symptoms or signs of cerebral lesions (MRI) or systemic metastasis (total body CT). CONCLUSION: In the literature the number of desmoplastic fibroma is too small and the follow-up period too short to permit any conclusions regarding the aggressiveness of the tumor.
BACKGROUND AND PURPOSE: We present a rare case of desmoplastic fibroma of the skull. Desmoplastic fibroma is a distinctive and rare neoplasm of bone that histologically and biologically mimics desmoid soft tissue tumor. Only 6 cases have been reported in the skull and none of these was diagnosed by MRI. CLINICAL PRESENTATION: A 64 year-old woman, operated on in June 1991 for left mastectomy due to a ductal adenocarcinoma and again in October 1994 for a left frontal metastatic adenocarcinoma, was admitted in our Department of Neurosurgery because a control cerebral MRI had detected a diploic lesion, isointense on T1-weighted images and hyperintense on T2, with moderate enhancement, localized in a right parietal site. Neurological examination was negative. The lesion was surgically removed and a cranioplasty was performed. Histological diagnosis was desmoplastic fibroma. Twelve months after treatment she has no neurological symptoms or signs of cerebral lesions (MRI) or systemic metastasis (total body CT). CONCLUSION: In the literature the number of desmoplastic fibroma is too small and the follow-up period too short to permit any conclusions regarding the aggressiveness of the tumor.
Authors: Soo-Han Yoon; Se Hyek Kim; Yong Sam Shin; Young-Whan Ahn; Kyung-Gi Cho; Ki Bum Lee; Ki Hong Cho Journal: Childs Nerv Syst Date: 2005-04-02 Impact factor: 1.475