McCune-Albright syndrome

Case contributed by A.Prof Frank Gaillard

Presentation

Raised growth hormone.

Patient Data

Age: 30 years
MRI

Grossly expanded osseous structures including the right squamotemporal petrous temporal bone, central and right sphenoid bone with complete obliteration of right maxillary sinus and sphenoid sinus and most of the right ethmoid air cells. This has characteristic appearances of fibrous dysplasia

Despite marked expansion of many osseous structures including the anterior clinoid and the orbital walls, there is no crowding of the right orbital apex and right-sided skull base foramina including the orbital fissures, optic canal and foramen rotundum are not significantly narrowed. No evidence of right optic nerve compression or atrophy.

The pituitary fossa is near normal in size despite fibrous dysplasia involving the surrounding osseous structures.

The posterior pituitary bright spot is normal and intact and infundibulum is midline. The right side of the sella contains a 5 mm hypo enhancing structure which could represent a small pituitary adenoma in this clinical context.

CT

Widespread ground-glass expansion of the right side of the skull and face consistent with fibrous dysplasia is noted.

CT

Bony expansion with ground-glass matrix involving the right third rib which is diffusely expanded is present. A small region of bony expansion and lucency in the right sixth rib and the seventh rib is only partially imaged. 

Case Discussion

This patient is known to have McCune-Albright syndrome. The presumed microadenoma has not yet been operated upon.

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Case information

rID: 65412
Published: 19th Jun 2019
Last edited: 17th Aug 2019
Inclusion in quiz mode: Included

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