105例口腔颅颌面部纤维性结构不良的临床病理分析

Clinicopathological analysis of 105 patients with fibrous dysplasia of cranio-maxillofacial region

目的:比较不同类型口腔颅颌面部纤维性结构不良(fibrous dysplasia,FD)的临床病理特点,为临床诊治及预后判断提供依据。方法:收集2013年1月至2020年12月于北京大学口腔医院确诊为FD或McCune-Albright综合征(McCune-Albright syndrome,MAS)的患者105例,分析其临床资料、影像学和病理学特点,分别归类为单骨型、多骨型、MAS型及口腔颅颌面部特有的口腔颅面型纤维性结构不良(craniofacial fibrous dysplasia,CFD)四型,对各型的临床病理特征、治疗及随访资料等进行分析。结果:105例患者的男女比为1∶1.3,发病年龄0~56岁(中位数为12岁)。本组病例中,单骨型43例(40.95%),其中上颌骨29例,下颌骨12例,颧骨2例;多骨型32例(30.48%);MAS型7例(6.67%);发生在颅上颌部位的FD,经影像学分析确诊CFD型23例(21.90%)。CFD在患者性别、术前血清碱性磷酸酶水平等方面有别于其他类型的FD。病理学上,各型FD的形态特点类似,但多骨型及MAS型患者可能出现以纤维成分增生为主的特征。结论:口腔颅颌面部FD的临床和病理特点有别于身体其他部位发生的FD病变,CFD的临床和病理特点与发生于颅颌面部的单骨型或多骨型FD也有显著差异,应注意区分,以进一步明确CFD在临床处置及预后等方面的特殊性。

Objective:To compare the clinicopathologic features and prognosis of the different types of fibrous dysplasia(FD)of cranio-maxillofacial region,so as to provide a new reference for clinicians to treat these patients and make prognostic judgement.Methods:Clinical records,radiographic data and pathological information of 105 patients diagnosed with FD or McCune-Albright syndrome(MAS)at the Department of Oral Pathology,Peking University Hospital of Stomatology from January 2013 to December 2020 were collected.The patients were divided into 4 groups:monostotic FDs,polyostotic FDs,MAS and a specific type called craniofacial fibrous dysplasia(CFD)limited in the craniofacial region.The clinicopathological characteristics,treatment and follow-up data of each type were analyzed.Results:Of all the 105 patients,46 were males and 59 were females,with a male-to-female ratio of 1∶1.3.The onset age ranged from 0 to 56 years and the median age was 12 years.On the basis of different involvement conditions,4 types were divided.The most common type was monostotic FDs(43 cases,40.95%),including maxilla(29 cases),mandibular(12 cases)and zygoma(2 cases).32 cases(30.48%)were diagnosed with polyostotic FDs,7 cases(6.67%)were MAS,and 23 cases(21.90%)were CFDs confirmed by computed tomography(CT)analysis.CFD was clearly distinct from other types of FD,such as the patient gender and the serum alkaline phosphatase level in peripheral blood before operative surgery.The pathologic findings of various types FD were quite similar,whilst the predominant fibrous tissue hyperplasia could be observed in polyostotic FDs and MAS types.Conclusion:The clinicopathologic features of FD in the cranio-maxillofacial region are different from the FD lesions in other parts of the body.The clinicopathological features of CFD are significantly different from those of monostotic and polyostotic FDs in the cranio-maxillofacial region.Therefore,the clinicians should pay attention to distinguish CFD in clinic,imaging and pathology aspects,so as to further clarify its features in clinic management and prognosis.