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 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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mccne - albright综合征合并肢端肥大症:1例具有纤维发育不良的影像学特征。
麦库恩-奥尔布赖特综合征(MAS)是一种罕见的多系统疾病,其临床特征是多骨纤维发育不良(FD)、皮肤色素沉着和功能亢进内分泌病。一名42岁男性因间歇性头痛来我院就诊,诊断为MAS伴肢端肥大症。该患者表现出多种MAS临床特征,包括垂体腺瘤、多囊性FD、促性腺功能减退。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病变,下颌骨混合透光-不透光的多室病变,中轴和阑尾骨骼透光病变。多年来,患者因FD的脆性骨性状态导致意外骨折多次住院。本报告提出了一个病例MAS的回顾性描述,并回顾了相关文献。
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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
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