105例颅颌面骨纤维异常增殖症患者的临床病理分析
[Clinicopathological analysis of 105 patients with fibrous dysplasia of cranio-maxillofacial region].
作者信息
Xue J, Zhang J Y, Shi R R, Xie X Y, Bai J Y, Li T J
机构信息
Department of Oral Pathology, Peking University School and Hospital of Stomatology & National Center of Stomatology & National Clinical Research Center for Oral Diseases & National Engineering Laboratory for Digital and Material Technology of Stomatology & Beijing Key Laboratory of Digital Stomatology, Beijing 100081, China.
Central Laboratory, Peking University School and Hospital of Stomatology & National Center of Stomatology & National Clinical Research Center for Oral Diseases & National Engineering Laboratory for Digital and Material Technology of Stomatology & Beijing Key Laboratory of Digital Stomatology, Beijing 100081, China.
出版信息
Beijing Da Xue Xue Bao Yi Xue Ban. 2022 Feb 18;54(1):54-61. doi: 10.19723/j.issn.1671-167X.2022.01.009.
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.
目的
比较颅颌面区域不同类型骨纤维异常增殖症（FD）的临床病理特征及预后，为临床医生治疗此类患者及进行预后判断提供新的参考依据。
方法
收集2013年1月至2020年12月在北京大学口腔医院口腔病理科确诊为FD或McCune-Albright综合征（MAS）的105例患者的临床记录、影像学资料及病理信息。将患者分为4组：单骨型FD、多骨型FD、MAS以及局限于颅面部的一种特殊类型即颅面部骨纤维异常增殖症（CFD）。分析各类型的临床病理特征、治疗及随访数据。
结果
105例患者中，男性46例，女性59例，男女比例为1∶1.3。发病年龄0～56岁，中位年龄12岁。根据受累情况不同分为4型。最常见的类型是单骨型FD（43例，40.95%），包括上颌骨（29例）、下颌骨（12例）和颧骨（2例）。32例（30.48%）诊断为多骨型FD，7例（6.67%）为MAS，23例（21.90%）经计算机断层扫描（CT）分析确诊为CFD。CFD与其他类型的FD明显不同，如患者性别及术前外周血血清碱性磷酸酶水平。各类型FD的病理表现较为相似，而多骨型FD和MAS类型中可见主要的纤维组织增生。
结论
颅颌面区域FD的临床病理特征与身体其他部位的FD病变不同。CFD的临床病理特征与颅颌面区域单骨型和多骨型FD有显著差异。因此，临床医生应在临床、影像学及病理方面注意鉴别CFD，以便在临床管理及预后方面进一步明确其特征。
Zotero 插件