Albright综合征恶变为上颌骨软骨肉瘤的首例报道

• 作 者: 郭丽敏
• 出 处: 国外医学(耳鼻咽喉科学分册)
• 发表日期: 2003年02期
• 关键词: 颌骨软骨肉瘤 Albright综合征 首例报道 骨纤维 发育异常 内分泌紊乱 恶变率 骨型 性早熟 上颌骨切除术

Albright综合征是多骨型骨纤维发育异常的一种类型。除了几个骨部的骨纤维发育异常外(多发生于颅面骨),还合并有皮肤色素沉着及性早熟,亦常有内分泌紊乱,如甲状腺功能亢进。文献报道中,单骨型及多骨型骨纤维发育异常的恶变发生率共约0.4％,而Al-bright综合征的恶变率则增至4％。本文首次报道1例发生于上颌骨的由Albright综合征恶变的软骨肉瘤。

Albright syndrome is a type of polypoid bone dysplasia. In addition to several bone bone fiber dysplasia (mostly occurs in the craniofacial bone), but also with skin pigmentation and precocious puberty, endocrine disorders are also often, such as hyperthyroidism. Reported in the literature, single and multiple bone dysplasia, the incidence of malignant transformation is about 0.4%, while Al-bright syndrome, the rate of malignancy increased to 4%. This is the first report of a case of chondrosarcoma in Albright’s syndrome that occurs in the maxilla.