Mesh : Female Humans Adult Craniofacial Fibrous Dysplasia Cross-Sectional Studies Fibrous Dysplasia, Monostotic / pathology Osteosarcoma Bone Neoplasms

来  源:   DOI:10.1016/j.oooo.2022.12.007

Abstract:
A systematic review was undertaken to examine the spontaneous of craniofacial fibrous dysplasia (FD) into osteosarcoma (OS).
Five databases were searched, and data were evaluated in 2 subsets: (1) frequency analysis involving only cross-sectional studies that specifically examined patients for osteosarcomatous transformation, thereby allowing a calculation of the frequency of transformation of craniofacial FD, and (2) case analysis of case reports and case series of FD that underwent osteosarcomatous transformation. Quality assessment of the studies in the frequency and case analyses was performed.
Seven cross-sectional studies involving 779 patients in the frequency analysis documented transformation of craniofacial FD into OS in 13 patients, for a frequency of 1.67%. Of the 18 separate cases of osteosarcomatous transformation in the case analysis, 11 (61.1%) occurred in females, 11 (61.1%) developed from monostotic FD, and 7 (38.9%) arose in the mandible, with 5 in the maxilla (27.8%) and 6 (33.3%) in other craniofacial sites. The mean age of the patients at the time of malignant diagnosis was 36.4 years, with an average of 11.3 years from FD diagnosis to the appearance of OS. The quality of the articles ranged from fair to excellent.
The risk of osteosarcomatous transformation of craniofacial FD is low, but the possibility of transformation should be taken into consideration by clinicians, as this will guide treatment and follow-up regimens.
摘要:
目的:进行了系统评价,以检查颅面纤维发育不良(FD)转化为骨肉瘤(OS)的自发性。
方法:搜索了五个数据库,和数据在2个子集进行了评估:(1)频率分析,仅涉及专门检查骨肉瘤转化患者的横断面研究,从而可以计算颅面FD的变换频率,(2)接受骨肉瘤转化的FD病例报告和病例系列的病例分析。在频率和病例分析中对研究进行质量评估。
结果:在频率分析中涉及779例患者的7项横断面研究记录了13例患者的颅面FD转变为OS,频率为1.67%。在病例分析的18例骨肉瘤性转化中,11例(61.1%)发生在女性中,11(61.1%)由单核FD发展而来,7(38.9%)出现在下颌骨中,上颌骨有5个(27.8%),其他颅面部位有6个(33.3%)。病人在恶性诊断时的平均年龄为36.4岁,从FD诊断到OS出现平均11.3年。文章的质量从一般到优秀不等。
结论:颅面FD骨肉瘤转化的风险较低,但是临床医生应该考虑转变的可能性,因为这将指导治疗和后续方案。
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