Imaging Sci Dent.  2022 Dec;52(4):421-427. 10.5624/isd.20220825.

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

• Yeom HG
• Lee BD

• KMID: 2537209
• DOI: http://doi.org/10.5624/isd.20220825

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.

Keyword

Fibrous Dysplasia; Polyostotic; Mandible; Acromegaly; Radiography