Abstract
BackgroundCraniomaxillofacial fibrous dysplasia (FD) is a benign bone lesion characterized by facial disfigurement and functional impairment. The aim of this study was to characterize the epidemiological and clinical features of craniomaxillofacial FD by presenting data from a representative Chinese population during a 15-year period (1994–2009).MethodThe craniomaxillofacial disease registries of two Chinese tertiary referral hospitals (Shanghai Ninth People’s Hospital and Stomatological hospital of Jiangsu Province) were searched and reviewed to collect relevant information for patients with craniomaxillofacial FD between Jan.1994 and Dec.2009. All included cases were further analyzed with regard to associated epidemiological and clinicopathological variables.ResultsA total number of 266 cases with definitive diagnosis were identified with 219 primary cases and 47 recurrent cases. There were 111 males and 155 females with a male to female ratio of 0.716:1. They were clinically categorized into three groups: monostotic (71.1%), polysotic (27.4%) and Albright syndrome (1.5%). Maxilla alone or with adjacent bones was the most common affected site. The serum alkaline phosphatase (ALP) in patients was much higher than that in healthy control, whereas comparable between primary patients and recurrent ones. Three patients (3/266, 1.1%) with polysotic lesions underwent spontaneous malignant transformation into osteosarcoma. The majority of patients underwent conservative surgery, while the others received radical resection with or without reconstruction.ConclusionsCraniomaxillofacial FD is a rare bony disorder with defined epidemiological and clinicopathological features in Chinese population. Further investigations are warranted to establish the optimized timing, treatment strategy and prognostic prediction for this clinical entity.
Highlights
Fibrous dysplasia (FD) is a benign bone lesion characterized by the replacement of normal bone structure with abnormal fibro-osseous connective tissue [1]
Craniomaxillofacial fibrous dysplasia (FD) usually begins in childhood and slowly progresses through puberty, resulting in devastating facial deformities and functional deficits [3]
We retrospectively reviewed and presented the epidemiological and clinical data of craniomaxillofacial FD from two Chinese tertiary referral hospitals over the past 15 years (1994–2009) in order to highlight the demographic, clinical and pathological characteristics in a large representative Chinese population
Summary
Fibrous dysplasia (FD) is a benign bone lesion characterized by the replacement of normal bone structure with abnormal fibro-osseous connective tissue [1]. It accounts for about 2-3% of bone-derived tumors and usually occurs throughout the skeletons around the body with a predilection for craniomaxillofacial bones [2]. Craniomaxillofacial FD usually begins in childhood and slowly progresses through puberty, resulting in devastating facial deformities and functional deficits [3]. These lesions develop in single bone (monstotic) and in multiple bones (polystotic), sometimes concomitantly associated with skin pigmentation and endocrine disturbances (McCune-Albright syndrome). The aim of this study was to characterize the epidemiological and clinical features of craniomaxillofacial FD by presenting data from a representative Chinese population during a 15-year period (1994–2009)
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
