McCune-Albright syndrome with acromegaly: A case report with characteristic radiographic features of fibrous dysplasia.

IF 1.7 Q3 DENTISTRY, ORAL SURGERY & MEDICINE Imaging Science in Dentistry Pub Date : 2022-12-01 Epub Date: 2022-10-12 DOI:10.5624/isd.20220825
Han-Gyeol Yeom, Byung-Do Lee
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Abstract

McCune-Albright syndrome (MAS) is a rare multisystem disorder characterized by a clinical triad of polyostotic fibrous dysplasia (FD), skin pigmentation, and hyperfunctioning endocrinopathies. A 42-year-old man visited our medical hospital for the treatment of intermittent headaches and was diagnosed with MAS with acromegaly. This patient showed various clinical features of MAS, including pituitary adenoma, polyostotic FD, and hypogonadotropic hypogonadism. The FD lesions showed characteristic radiographic features, such as widespread, sclerotic bony lesions in the cranial bones, mixed radiolucent-radiopaque multilocular lesions in the mandible, and radiolucent lesions in the axial and appendicular skeleton. Over the years, the patient had been hospitalized multiple times due to accidental bony fractures associated with the fragile bony state of FD. This report presents a retrospective description of a case of MAS, with a review of the relevant literature.

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麦库恩-阿尔布莱特综合征伴肢端肥大症:具有纤维发育不良放射学特征的病例报告。
麦库恩-阿尔布莱特综合征(McCune-Albright Syndrome,MAS)是一种罕见的多系统疾病,临床特征为多发性骨纤维发育不良(FD)、皮肤色素沉着和内分泌功能亢进三联征。一名 42 岁的男子因间歇性头痛到我院就诊,被诊断为 MAS 并发肢端肥大症。该患者表现出MAS的各种临床特征,包括垂体腺瘤、多发性FD和性腺功能减退症。FD病变表现出特征性的影像学特征,如颅骨广泛的硬化性骨质病变、下颌骨放射性-不透明多形性混合病变以及轴向和阑尾骨骼的放射性病变。多年来,由于 FD 骨质脆弱,患者曾多次因意外骨折住院治疗。本报告对一例 MAS 病例进行了回顾性描述,并对相关文献进行了综述。
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来源期刊
Imaging Science in Dentistry
Imaging Science in Dentistry DENTISTRY, ORAL SURGERY & MEDICINE-
CiteScore
2.90
自引率
11.10%
发文量
42
期刊最新文献
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