Fibrous Dysplasia

 However, matrix may range from purely lytic to purely sclerotic lesion



• Narrow zone of transition ± sclerotic margin

• Low to intermediate signal intensity on T1WI, heterogeneous on T2WI, STIR, variable enhancement

• Mild to marked increase in radionuclide uptake




Top Differential Diagnoses




• Aneurysmal bone cyst (ABC)

• Paget disease

• Osteoblastoma

• Osteosarcoma

• Tuberous sclerosis


Pathology




• Sporadic mutation in GNAS  gene


Clinical Issues




• Growth disturbance, pathologic fracture

• Rarely undergoes sarcomatous transformation


Diagnostic Checklist




• Do not confuse with Paget disease on imaging
image Paget disease thickens cortex and trabeculae

image FD thins cortex and replaces trabeculae

image
(Left) Sagittal bone CT shows severe polyostotic fibrous dysplasia (FD) involving skull, facial bones, and cervical spine. Some areas are “ground-glass” image, others are purely lytic image, and there are a few foci of calcified cartilage image.


image
(Right) Coronal bone CT in the same patient shows loss of normal trabeculae in the majority of the included bones, replaced by fibrous dysplasia matrix. Thinning of tables of skull is a characteristic finding.

image
(Left) Axial bone CT shows variation in density image in a single vertebra with nearly complete marrow replacement. This variability is common and should not raise suspicion for malignant degeneration.


image
(Right) Axial bone CT shows lytic FD image in the posterior elements. The narrow zone of transition image (sometimes sclerotic) helps to distinguish FD from more aggressive processes. Vertebral bodies tend to be less severely involved than the posterior elements.


TERMINOLOGY


Abbreviations




• Fibrous dysplasia (FD)


Definitions




• Monostotic: Single lesion

• Polyostotic: Multiple lesions, often associated with growth disturbances

• McCune-Albright syndrome: Polyostotic FD, precocious puberty, café au lait skin lesions


IMAGING


General Features




• Best diagnostic clue
image Expansile lesion with “ground-glass” bone matrix

• Location
image Rarely involves spine
– Most spine lesions occur in patients with polyostotic disease

– Neural arch > vertebral body

image Commonly involves pelvis
– Innominate bone much more frequently involved than sacrum


Radiographic Findings




• Radiography
image Fusiform expansion of bone

image Cortical thinning

image Commonly ground-glass matrix
– However, matrix may range from purely lytic to purely sclerotic lesion

– May contain islands of cartilage

image Narrow zone of transition ± sclerotic margin

image Polyostotic lesions tend to predominate on 1 side of body

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Oct 5, 2016 | Posted by in NEUROLOGICAL IMAGING | Comments Off on Fibrous Dysplasia

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