Desmoplastic Fibroma of the Cranium: Case Report and Review of the Literature

Abstract

Desmoplastic fibroma of the cranium is rare. Surgical resection is the treatment of choice.

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.…”

“…Infiltrative growth in the soft tissue was documented in 48% of patients [1]. DFs of the cranium are typically solitary, lytic lesions of a honeycomb or trabeculated pattern, with a mild or absent sclerotic reaction at the margins [9,17]. Typical CT findings are destruction and thinning of the bony cortex, and MRI can show the intermediate intensity on T1-weighted images and the heterogeneous intensity on T2-weighted images [17].…”

“…DFs of the cranium are typically solitary, lytic lesions of a honeycomb or trabeculated pattern, with a mild or absent sclerotic reaction at the margins [9,17]. Typical CT findings are destruction and thinning of the bony cortex, and MRI can show the intermediate intensity on T1-weighted images and the heterogeneous intensity on T2-weighted images [17]. In our patient, the radiological differential diagnosis based on the preoperative films was fibrous dysplasia, desmoplastic fibroma, chondromyxoid fibroma or nonossifying fibroma.…”

“…Temporal bone [17] Female/43 Temporoparietal bone [7] Female/7 Frontal bone [15] Male/3 month Parietal bone This study Female/2 Frontotemporal bone diffusely hyalinized (Fig. 5c).…”

“…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].…”