Primary musculoskeletal tumors of fibrous origin

Abstract

Tumors of fibrous origin include fibrous dysplasia (FD), fibroxanthoma (nonossifying fibroma), cortical desmoid, desmoplastic fibroma, fibrosarcoma, and malignant fibrous histiocytosis (MFH). Benign fibrous lesions (FD, fibroxanthoma, and cortical desmoid) frequently demonstrate pathognomonic radiologic characteristics obviating the need for biopsy. Indeed, biopsy of these lesions can occasionally lead to confusion with more aggressive lesions. Desmoplastic fibroma and the malignant fibrous lesions (fibrosarcoma and MFH) often reveal nonspecific imaging features of a solitary nonmineralized lesion with aggressive characteristics. However, imaging is important as with other neoplasms in delineating the extent of involvement for staging purposes. This article reviews the spectrum of clinical characteristics, pathology, imaging appearances, treatment, and prognosis of lesions of fibrous origin in bone.