【摘要】 颌骨中央性巨细胞病变(CGCL)相对少见,其病因、发病机制、疾病性质尚不明确。CGCL可能是由之前的创伤和骨内出血而诱发的反应性肉芽肿性修复过程。由于CGCL可发生于已知的遗传病(如Ⅰ型神经纤维瘤病、Noonan综合征等)患者,故基因相关的病因学可能在疾病发生中发挥了作用。综合征患者颌骨CGCL的表型可能与Ras/丝裂原活化蛋白激酶信号通路改变有关。巨细胞肿瘤存在H3F3A基因点突变,但散发的颌骨CGCL并不具有该特征,提示颌骨CGCL与颌骨外巨细胞肿瘤并非同一疾病。更多还原

【Abstract】 Central giant cell lesions( CGCL) of the jaws are relatively uncommon. The etiology,true nature and pathogenesis of the lesions have not been clarified. It was suggested that CGCL could be the result of an reparative process related to previous trauma and an intraosseous hemorrhage which had triggered the reactive granulomatous process. The occurrence of CGCL in the jaws of patients with known genetic diseases,such as neurofibromatosis type Ⅰ,and Noonan syndrome,indicates that a genetic-related etiology might be possible. The phenotype of CGCL of the jaws in syndromic patients may be the result of dysregulation of Ras /MAPK signaling. The somatic mutations in the H3F3 A gene were found in patients with extragnathic giant cell tumor,however sporadic CGCL of the jaws did not share the H3F3 A mutations,which indicated that the CGCL of the jaws was distinct and separate from the extragnathic giant cell tumor.更多还原