McCune-Albright syndrome

McCune-Albright

syndrome • McCune-Albright syndrome - fibrous dysplasia - Ganzer Fall bei Radiopaedia

Fibrous

dysplasia for radiologists: beyond ground glass bone matrix. Bilateral ovarian cysts in McCune-Albright syndrome (red arrows)

McCune

Albright Syndrome (MAS) – polyostotic fibrous dysplasia. Conventional radiography of the right femur (AP projection) shows a slightly expansile lesion (asterisk) in the proximal diaphysis with a ground-glass appearance and widening of the medullary cavity. Note subtle deformity of the hip (arrows).

McCune

Albright Syndrome (MAS) – polyostotic fibrous dysplasia. MR of the brain. The mid-sagittal T2- weighted image demonstrate marked thickening of frontal-, occipital-, sphenoid bone and clivus. Intracranial structures are normal.

McCune

Albright Syndrome (MAS) – polyostotic fibrous dysplasia. MR of the brain. Axial T2-weighted images (a), FLAIR images (b). MR shows marked but asymmetrical thickening of the calvaria. Although the lesions are slightly inhomogeneous, the overall appearance is of predominant low signal.

McCune

Albright Syndrome (MAS) – polyostotic fibrous dysplasia. Bone scintigraphy. Anterior (a) and posterior (b) projection show increased osteoblastic activity in the skull, ribs, pelvis, left humerus, both femora, right tibia and talus.

McCune

Albright Syndrome (MAS) – polyostotic fibrous dysplasia. Conventional radiography of the right foot (oblique projection) depicts the ground-glass appearance in the talar body and neck (asterisk) and deformity of the head (arrowheads).

McCune

Albright Syndrome (MAS) – polyostotic fibrous dysplasia. Conventional radiography of the right foot (AP projection) depicts a focal radiolucent lesion with sclerotic margin in the talar head (arrows). Note also lesions with ground-glass aspect in the metatarsals (thick open arrows).

McCune

Albright Syndrome (MAS) – polyostotic fibrous dysplasia. Conventional radiography of the left humerus (AP projection) shows bone expansion of the proximal diaphysis of the left humerus with homogeneous ground-glass matrix (arrows). Note sclerotic teeth-like border with normal proximal metaphysis (arrowheads).

McCune

Albright Syndrome (MAS) – polyostotic fibrous dysplasia. Conventional radiography of the left femur (AP projection) shows a ground-glass lesion in the proximal diaphysis. Note also more subtle lesions in the middle and distal diaphysis (arrowheads). Note slight deformity of the femoral diaphysis.

McCune-Albright

syndrome • McCune-Albright syndrome - Ganzer Fall bei Radiopaedia

McCune-Albright

syndrome • McCune-Albright syndrome - Ganzer Fall bei Radiopaedia

Fibrous

dysplasia for radiologists: beyond ground glass bone matrix. Testicular abnormalities in McCune-Albright syndrome in three different patients. a, b Ultrasound (US) of the testicles shows extensive echogenic material secondary to Leydig cell hyperplasia. c, d The heterogeneous appearance of the testicle with more focal, well-circumscribed areas of abnormal echogenicity. These imaging findings correlate with Leydig cell hyperplasia on pathology. e, f The right testicle appears atrophic and demonstrates inhomogeneous echotexure and multiple punctate calcifications

McCune-Albright

syndrome • Fibrous dysplasia - polyostotic form - Ganzer Fall bei Radiopaedia

McCune-Albright

syndrome • Fibrous dysplasia in McCune-Albright syndrome - Ganzer Fall bei Radiopaedia

McCune-Albright

syndrome. CT for detecting subtle fractures. A) This radiograph was from a 9-year-old boy who complained of new-onset, focal pain in the groin. No discernable fracture is apparent. B) Reformatted CT images of the lesion revealed a fracture in the medial proximal femur (arrows).

McCune-Albright

syndrome. Fluid-filled cyst in FD. True fluid-filled cysts can occur in FD lesions. Shown is the CT scan of fluid-filled cyst that arose in a 12 year old boy with MAS who presented with facial nerve parasthesias and displacement of the orbit that occurred over approximately one week. The fluid-filled nature of the lesion is demonstrated by the fluid-fluid level (arrow). These lesions are more frequent in the craniofacial bones and can be aggressive. They usually require prompt surgical intervention.

McCune-Albright

syndrome. Radiographic appearance of fibrous dysplasia (FD). A) A proximal femur with typical ground glass appearance and shepherd"s crook deformity in a 10-year-old child is shown. B) The appearance of FD in the femur of an untreated 40-year-old man demonstrates the tendency for FD to appear more sclerotic with time C) The typical ground glass appearance of FD in the craniofacial region on a CT image of a 10-year-old child is shown. The white arrows indicate the optic nerves, which are typically encased with FD. D) A CT image in a 40-year-old woman demonstrates the typical appearance of craniofacial FD in an older person, with mixed solid and "cystic" lesions. The Hounsfield Unit measurements of "cystic" lesions are quite useful in distinguishing soft tissue "cystic" lesions from true fluid-filled cysts, which are much more uncommon and tend to behave aggressively with rapid expansion and compression of vital structures. E-G) Bone Scintigraphy in FD. Representative 99Tc-MDP bone scans which show tracer uptake at affected skeletal sites, and the associated skeletal disease burden score (see ref. Collins, 2005) are shown. E) A 50-year-old woman with monostotic FD confined to a single focus involving contiguous bones in the craniofacial region. F) A 42-year-old man with polyostotic FD shows the tendency for FD to be predominantly (but not exclusively) unilateral, and to involve the skull base and proximal femur. G) A 16-year-old boy with McCune-Albright syndrome and involvement of virtually all skeletal sites (panostotic) is shown [65].

Fibrous

dysplasia for radiologists: beyond ground glass bone matrix. Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas in McCune-Albright syndrome (blue arrows). T2-weighted MRI (a), T1 contrast-enhanced MRI (b). Coronal maximum intensity projection from a 3D T2-weighted MRCP acquisition shows IPMNs (c). Please note, a fibrous dysplasia lesion in the left rib (green arrows)

Fibrous

dysplasia for radiologists: beyond ground glass bone matrix. Thyroid abnormalities in McCune-Albright syndrome (MAS) in three different patients. a–c Ultrasound (US) of the thyroid gland shows typical microcystic changes. Macrocystic pattern (d–f) and solid thyroid nodules (g–i) can also be seen in patients with MAS

McCune

Albright Syndrome (MAS) – polyostotic fibrous dysplasia. MR of the brain. The axial T2 weighted image (a) show narrowing of the orbit. There is no definite compression of the optic nerve (arrow) on the coronal image (b).