Abstract
Desmoplastic fibroma, also known as desmoid fibroma of bone, is a rare benign bone tumor, accounting for approximately 0.1 to 0.3% of all bone tumors. It was first described by Jaffe in 1958. This tumor has a slow progression with locally aggressive behavior, without metastasis or malignant transformation. For radiological diagnosis, magnetic resonance imaging (MRI) is the modality of choice to assess local extension within the bone and, when present, into the surrounding soft tissues. The definitive diagnosis is histological: the tumor is composed of tissue poor in fibroblasts and rich in collagen fibers, identical to the histology of desmoid fibromas of the soft tissues. Histological differential diagnosis can be challenging, particularly with low-grade fibrosarcomas. The treatment of choice is surgical, consisting of wide tumor resection whenever the location allows. A high rate of local recurrence characterises post-treatment evolution in cases of intralesional excision.