Desmoplastic fibroma of the bone: A report of two patients, review of the literature, and therapeutic implications

Abstract

Considering the "semimalignant" character of this entity and the poor treatment results in patients with recurrent tumors, marginal or wide resection for primary treatment is recommended. The superior imaging quality of MRI greatly facilitates preoperative planning.

Desmoplastic fibroma of the skull in an infant

“…However, proliferation indexes from flow cytometric DNA analysis for locally infiltrating DFs in two patients were reported to be between 21.5 and 24% [1]. Tumor cell staining with MIB-1 was reported in two patients: less than 5% in one patient, and 20% in other [1]. No cases reported a proliferation index in the previous literature on cranial DFs, but the present case showed a few cells with MIB-1 staining in high-power fields.…”

“…A lack of mitotic figures and nuclear atypia may distinguish DF from malignant bone neoplasms [4,17]. However, proliferation indexes from flow cytometric DNA analysis for locally infiltrating DFs in two patients were reported to be between 21.5 and 24% [1]. Tumor cell staining with MIB-1 was reported in two patients: less than 5% in one patient, and 20% in other [1].…”

“…It occurs equally in both sexes, and three-quarters arise during the first three decades of life, with a mean age of 23 years [1,3,9]. DFs are found most commonly in the metaphyses of the long bones [1,3,18], and the mandible [7], but rarely in the maxilla, calvaria, sternum, vertebrae, and parapharyngeal space [6,8,9,13,20].…”

“…The cut section of the mass shows a solid fibrous surface with focal hemorrhage Radiographically, DF in the long bones is characterized by local expansile lytic lesion in the metaphysis with thinning of the cortex, and a soap bubble appearance or diffuse trabeculation without periosteal reaction [12]. 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].…”

“…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]. We present the 12th known case of DF of the skull, which is the second known infantile DF of the skull.…”