Imaging of Fibrous Dysplasia: A Comprehensive In-Depth Analysis of Monostotic, Polyostotic, Syndromic Forms, and Bone...

Fibrous dysplasia is one of the most common skeletal lesions. The wide spectrum of clinical manifestations ranges from asymptomatic conditions (typical of monostotic forms) to severe skeletal diseases with deformity and fractures for polyostotic fibrous dysplasia. The classical radiological features include: an osteolytic geographic pattern, ground-glass bone matrix, cortical thinning/cortical scalloping, bone deformities and enlargement, concavity of margins (evaluated with MRI), and cystic areas (MRI). All the bones can be affected, and the proximal femur is the most common one (about 30% of cases). Nonetheless, the disease can also affect cranio-facial bones, leading to compression of neural structures, as well as deformation and enlargement of facial bones, leading to the so-called "leontiasis ossea" or "facies leonine". The polyostotic forms of fibrous dysplasia can be associated with multiple soft-tissue myomas (Mazabraud syndrome) or several endocrine diseases (McCune-Albright syndrome). In every diagnostic step of the disease, as well as in different fibrous dysplasia forms, imaging plays a key role. Indeed, radiology is fundamental to assess the suspicion of fibrous dysplasia in classical monostotic forms, representing the sole diagnostic tool needed in many cases. Imaging is also fundamental to staging and following up on more severe polyostotic forms, as well as for detecting complications. In this comprehensive updated review article, we examine every aspect of the disease, with a main focus on imaging presentation. The indications for biopsy are discussed as well. Most importantly, the article details the potential risk of malignant transformation (osteosarcoma, fibrosarcoma, chondrosarcoma, and other rarer sarcomas, all accounting for <1% of cases) underlying the radiological patterns of these conditions. The occurrence of aneurysmal bone cyst-like changes on fibrous dysplasia is also analyzed in the article. This review article aims to be a comprehensive guide for radiologists and clinicians involved in the care of patients affected by various forms of fibrous dysplasia, and a starting point for future research. Many classical and atypical cases are collected as an iconographic comprehensive representation.