Fibrous Dysplasia of the Jaw: Advances in Imaging and Treatment

Abstract

A total of 7% of all benign bone lesions are diagnosed as fibrous dysplasia (FD). The symptoms of FD of the jaw range from asymptomatic to dental anomalies, pain and facial asymmetry. Due to its resemblance to other fibro-osseous bone lesions, misdiagnosis often occurs and can lead to inadequate treatment. Particularly in the jaw, this lesion does not become quiescent during puberty, making fundamental knowledge about the diagnosis and treatment of FD crucial. Mutational analysis and nonsurgical approaches offer new diagnostic and therapeutic options. In this review, we examine the advances and the difficulties of the diagnosis and the various treatment modalities of FD of the jaw in order to capture the current scientific knowledge on this bone disease.

A Case Series and Literature Review of Craniofacial Fibrous Dysplasia

“…FD accounts for around 7% of benign bone diseases and 2.5% of all bone lesions [ 6 ]. It affects approximately 1:30,000 individuals [ 10 ].…”

“…FD is radiologically and histologically distinct from other bones, presumably as a result of its desmal origin [ 6 ]. Histopathological diagnosis of FD is particularly challenging because of the overlapping characteristics with other fibro-osseous tumours [ 6 ].…”

“…FD is radiologically and histologically distinct from other bones, presumably as a result of its desmal origin [ 6 ]. Histopathological diagnosis of FD is particularly challenging because of the overlapping characteristics with other fibro-osseous tumours [ 6 ]. Three unique patterns can be seen at the microscopic examination: the hypercellular type, the Pagetoid type, and the conventional Chinese character shape [ 1 ].…”

“…The aetiology is linked to a mutation in the alpha-subunit (Gs-alpha) of the G signalling coupling protein expressed by gene GNAS (guanine nucleotide binding protein alpha stimulating) [ 5 ]. The disease has been categorized as quiescent, nonaggressive or aggressive based on their clinical characteristics [ 6 ]. Bone pain and recurrent fractures are the most prevalent manifestations during childhood and adolescence, subsequently followed by malformation and neurologic compression, particularly when the skull and face bones are affected [ 3 ].…”

“…Bone pain and recurrent fractures are the most prevalent manifestations during childhood and adolescence, subsequently followed by malformation and neurologic compression, particularly when the skull and face bones are affected [ 3 ]. Malignant transformation occurs in 0.4% to 4% of cases [ 6 ]. FD is diagnosed based on clinical, radiographic, and histological findings [ 7 ].…”