EURORAD ESR

Case 14477

McCune Albright Syndrome (MAS) – polyostotic fibrous dysplasia

Author(s)
Lukasz Augsburg1, Filip M. Vanhoenacker 1, 2, 3, Jan Gielen1

(1) University Hospital Antwerp,
Department of Radiology,
B- 2650 Antwerp, Belgium;
Email: filip.vanhoenacker@telenet.be
(2) AZ Sint-Maarten Duffel-Mechelen,
Department of Radiology;
2800 Mechelen, Belgium
(3) Faculty of Medicine and Health Sciences,
Ghent University,
Belgium
 
Patient
male, 15 year(s)
 
 
  • Figure 1
    Café au lait spots on the patient's back

    Irregularly delineated café-au-lait spots, resembling the coast of Maine in the United States of America.

     
    Area of Interest: Musculoskeletal system; Imaging Technique: Plain radiographic studies; Procedure: Education; Special Focus: Congenital;
     
     
  • Figure 2
    Conventional radiography of the right femur (AP projection)

    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...

     
    Area of Interest: Musculoskeletal bone; Imaging Technique: Conventional radiography; Procedure: Education; Special Focus: Congenital;
     
     
  • Figure 3
    Conventional radiography of the left femur (AP projection)

    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...

     
    Area of Interest: Musculoskeletal bone; Imaging Technique: Conventional radiography; Procedure: Education; Special Focus: Congenital;
     
     
  • Figure 4
    Conventional radiography of the right humerus (AP projection)

    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...

     
    Area of Interest: Musculoskeletal bone; Imaging Technique: Conventional radiography; Procedure: Education; Special Focus: Congenital;
     
     
  • Figure 5
    Conventional radiography of the right foot (AP projection)

    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...

     
    Area of Interest: Musculoskeletal bone; Imaging Technique: Conventional radiography; Procedure: Education; Special Focus: Congenital;
     
     
  • Figure 6
    Conventional radiography of the right foot (oblique projection)

    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).

     
    Area of Interest: Musculoskeletal bone; Imaging Technique: Conventional radiography; Procedure: Education; Special Focus: Congenital;
     
     
  • Figure 7
    Bone scintigraphy

    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.

     
    Area of Interest: Musculoskeletal bone; Imaging Technique: SPECT; Procedure: Education; Special Focus: Metabolic disorders;
     
     
  • Figure 8
    Magnetic resonance (MR) of the brain

    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...

     
    Area of Interest: Musculoskeletal joint; Imaging Technique: MR; Procedure: Education; Special Focus: Congenital;
     
     
  • Figure 9
    Magnetic resonance (MR) of the brain

    MR of the brain. The mid-sagittal T2- weighted image demonstrate marked thickening of frontal-, occipital-, sphenoid bone and clivus. Intracranial structures are normal.

     
    Area of Interest: Musculoskeletal bone; Imaging Technique: MR; Procedure: Education; Special Focus: Congenital;
     
     
  • Figure 10
    Magnetic resonance (MR) of the brain

    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).

     
    Area of Interest: Musculoskeletal bone; Imaging Technique: MR; Procedure: Education; Special Focus: Congenital;
     
     
Irregularly delineated café-au-lait spots, resembling the coast of Maine in the United States of America.
 
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).
 
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.
 
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).
 
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).
 
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).
 
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.
 
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.
 
MR of the brain. The mid-sagittal T2- weighted image demonstrate marked thickening of frontal-, occipital-, sphenoid bone and clivus. Intracranial structures are normal.
 
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).
 
 
 
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