Desmoplastic fibroma of the pediatric skull

Report of three cases

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✓Desmoplastic fibromas are rare bone tumors that have been reported in the adult skull but rarely in that of children. Although desmoplastic fibromas of the pediatric skull are uncommon, their similarity to benign skull lesions and their locally aggressive nature make them an important part of the differential diagnosis. Local recurrence is common after curettage alone but complete resection appears to be curative. Close follow up of incompletely resected lesions is essential. The authors detail three cases of pediatric desmoplastic fibromas of the skull and discuss diagnosis and treatment.

Article Information

Address reprint requests to: Greg Olavarria, M.D., 3200 SW 60th Court, MAB 301, Miami, Florida 33155. email: GOlavarria@med.miami.edu.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 2. Bone-window (A) and contrast-enhanced (B) CT scans demonstrating a lytic lesion eroding the outer table, with enhancement of an intralesional soft-tissue mass, typical of desmoplastic fibroma of the skull.

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    Case 3. A Gd-enhanced T1-weighted axial MR image of a well-defined soft-tissue mass expanding the diploic space with prominent contrast enhancement. A flow void related to the increased vascularity of this lesion is visible. There is no dural enhancement or parenchymal invasion.

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    Case 3. Intraoperative photograph illustrating erosion through the outer table of the right frontal skull. A craniectomy with grossly normal margins and split graft cranioplasty was performed.

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    Case 2. Photomicrograph showing hypocellular fibrous tissue in a storiform pattern interspersed with lakes of hyalinized collagen. H & E, original magnification × 200.

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