皮肤色素沉着-性早熟-进展性骨损害  

Skin pigmentation-precocious puberty-progressive bone lesion

作　　者：王亚冰[1] 王文博[1] 姜艳[1] 李梅[1] 夏维波[1] 邢小平[1] 王鸥[1] 孟迅吾[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院内分泌科国家卫生和计划生育委员会内分泌重点实验室,北京100730

出　　处：《中华骨质疏松和骨矿盐疾病杂志》2017年第3期277-283,共7页Chinese Journal Of Osteoporosis And Bone Mineral Research

基　　金：国家自然科学基金(81100559;81270873);国家临床重点专科建设项目(WBYZ2011-873)

摘　　要：骨纤维异常增生症(fibrous dysplasia,FD)是一种罕见的良性骨病,McCune Albright综合征(McCune Albright syndrome,MAS)为其特殊类型,主要表现为皮肤色素沉着、骨畸形、内分泌腺体功能异常三联征。FD恶变的发生率低但预后差,其主要病理类型为骨肉瘤。本文报道1例MAS年轻女性患者,其下颌骨FD病变在短时间内进行性疼痛、肿胀加重,最终手术病理证实为骨肉瘤,旨在提醒临床医生当FD患者出现上述病情变化时需高度警惕恶变可能。Fibrous dysplasia （FD） is a rare benign bone disease. McCune Albright syndrome （MAS） is a special FD type, mainly characterised by the clinical triad of skin pigmentation, bone deformities, and endocrine dysfunction. The incidence of malignant FD is low while the prognosis is poor, of which the main histologic type is osteosarcoma. Here in we reported a young woman who was diagnosed as MAS. Her FD lesion of the mandible was progressively painful and swelling within a short time. Eventually postoperative histopathology confirmed an osteosarcoma. We aimed to remind the clinicians of FD mafignant change when above-mentioned changes emerged.

关 键 词：骨纤维异常增生症 Mc Cune ALBRIGHT综合征 恶变 骨肉瘤 

分 类 号：R681[医药卫生—骨科学]