Desmoplastic fibroma of the skull

Abstract

Desmoplastic fibroma is a rare benign neoplasm of bone; only three cases have been reported involving the skull. The radiographic and histological aspects of a case of desmoplastic fibroma involving the parietal bone are reported.

Desmoplastic fibroma of the frontal bone

“…[1] Rarely the tumor occurs in the skull bones. [2][3][4][5][6] Majority have occurred in female adult patients, while one case of congenital desmoplastic fibroma was reported in a male child. [3] The duration of symptoms ranged from 1 month to 12 years and the age group ranged from 3 months to 86 years.…”

Desmoplastic fibroma of the skull in an infant

“…Most of the adult patients complained of headaches or cranial asymmetry, but the two girls and one infant boy presented only with a cranial mass. The time between symptom onset and initial presentation varied between several weeks and 12 years: 3 patients had symptoms for less than 6 months, 6 patients for more than 6 months, and 3 were discovered incidentally [2,4,7,9,10,[14][15][16][17]19]. Fig.…”

“…The small exposed area of the dura was managed with electric cauterization and chemical cauterization, and the skull defect was covered with a resin cranioplasty. In previously reported cases, most lesions were found to be substantially smaller, with no mention of the details pertaining to the management of the dura [2,7,9,10,[14][15][16]19], except in two recent cases [6,17]: one with electro-cauterization, and in the other dural incision was performed and the tumor was removed with the underlying dura. All 12 patients with cranial DFs did not have aggressive resection for the underlying dura, except for one patient, but they did not show any evidence of recurrence from possible dural tumor infiltration.…”

“…b MRI study of the head revealed a mass of low signal intensity on T1-weighted spin-echo images, and profoundly low signal intensity on T2-weighted fast spin echo images. The mass enhanced heterogeneously with gadolinium, with strong enhancement along the dural and bony margins of the mass, but weak enhancement of the center of the mass [14] Female/86 Temporal bone [9] Female/42 Supraorbital frontal bone [19] Female/28 Parietal bone [4] Female/72 Temporal bone [2] Female/64 Parietal bone [16] Female/21 Temporal bone Female/28…”

“…More than 200 cases of DFs have been reported, and are especially frequent in the long bones and mandible [3,5,12]. However, it is so rare in the cranium that only 11 cases of cranial DFs have been reported in the literature [2,4,7,9,10,[14][15][16][17]19] (Table 1): 1 case in an infant, 1 case in a child, and 9 cases in adults. Although DFs may radiologically mimic other, more common central nervous system neoplasms, the histological and biological features are characteristic [1,3,7,18].…”