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目的:探讨牙骨质骨化纤维瘤(COF)的遗传性、临床特征、诊断与治疗方法。方法:报道1例遗传性双侧上下颌骨巨大COF患者,并复习国内外的相关文献。结果:COF存在染色体缺失畸变,青年好发,常缓慢无痛性颌面部隆起,CT扫描显示低密度或混合密度影,边界清晰。病理示瘤组织由丰富的纤维组织细胞及散在分布的圆形或卵圆形的牙骨质小体及牙骨质样物质组成。结论:单侧颌骨COF报道较多,但双侧上下颌骨及遗传性发病非常罕见。COF可能为X-连锁隐性遗传。诊断主要依靠临床表现、CT及病理检查。治疗以手术切除为主,但术中易大出血。
Objective: To investigate the hereditary, clinical characteristics, diagnosis and treatment of osteofibroma fibroma (COF). Methods: One case of hereditary bilateral maxillary and maxillofacial COF was reported and related literatures at home and abroad were reviewed. Results: There was chromosome aberration and distortion in COF. The young patients developed normal and slow painless maxillofacial bulge. The CT scan showed low density or mixed density, and the border was clear. Pathology showed tumor tissue by a large number of fibrous tissue cells and scattered in the round or oval-shaped cementum body and cementitious material composition. Conclusion: There are many reports of unilateral maxilla COF, but the bilateral maxilla and mandible and genetic disease is very rare. COF may be X-linked recessive inheritance. The diagnosis depends mainly on clinical manifestations, CT and pathological examination. Surgical removal of the main treatment, but intraoperative bleeding.