Fibröse Dysplasie des Femur

Figuur 2:

Röntgenfoto’s van het proximale femur (heup) welke is aangedaan door fibreuze dysplasie. Op foto A is een normale weergave van proximale femur te zien. Op foto B is te een fibreuze laesie met typisch matglas aspect en afwezige weke delen reactie te zien. De vorm van het femur is op deze foto echter nog normaal. Op foto C is de herdersstaf afwijking te zien die typisch is voor een ernstige fibreuze dysplasie met herhaaldelijke (micro) fracturen waardoor het proximale femur in een bocht is gaan groeien. Die bocht wordt vaak vergeleken met de bocht in een herdersstaf.

Fibrous

dysplasia for radiologists: beyond ground glass bone matrix. The radiographic appearance of fibrous dysplasia (FD) and the rind sign. a–e Frontal radiographs demonstrate classic FD lesions in appendicular skeleton. A classic lucent lesion surrounded by a layer of sclerotic reactive bone (so-called the rind sign). The rind sign is most commonly seen in the proximal femur (red arrow)

Fibrous

dysplasia for radiologists: beyond ground glass bone matrix. MRI in fibrous dysplasia (FD). a, b MRI typically shows sharply demarcated lesions with intermediate to low signal intensity on T1-weighted images (WI) and intermediate to high on T2-WI (red arrow). c Some FD lesions may also contain small cystic areas, which make the T2 signal bright (green arrow). d FD lesions usually show some degree of enhancement after contrast administration (blue arrow)

Fibrous

dysplasia for radiologists: beyond ground glass bone matrix. “Evolution” of the fibrous dysplasia (FD) lesions. a Radiograph of a 3-year-old demonstrates a typical heterogeneous-appearing FD lesion in the femur. b Radiograph from an 11-year-old demonstrates homogeneous and radiolucent FD lesion. c Image from a 54-year-old patient shows sclerotic FD lesions

Fibrous

dysplasia for radiologists: beyond ground glass bone matrix. Fractures in fibrous dysplasia (FD). a Fractures are more frequent in childhood, with the highest rate occurring between 6 and 10 years of age. b, c Radiograph and CT of the left femoral bone demonstrate a fracture in the medial proximal femur in the settings of FD (green arrows) (reprinted from Dumitrescu and Collins [35])

Fibrous

dysplasia for radiologists: beyond ground glass bone matrix. The classification of femur deformities in fibrous dysplasia (FD). a Type 1. The neck-shaft angle is within normal limits (135°), but a distal femur shows 16° valgus deformity. b Type 2. The neck-shaft angle is valgus (152°). c Type 3. The neck-shaft angle is varus (100°). A distal shaft 10° demonstrates varus deformity. Distal juxta-articular valgus deformity is also present. d Type 4. The neck-shaft angle is normal (125°). Proximal lateral (shepherd’s crook) and distal medial bowing of the femoral shaft are present. e Type 5. The neck-shaft angle is valgus (160°). Lateral bowing of the proximal femur (shepherd’s crook) and medial bowing of the distal femur are present. f Type 6. FD affects the entire femur. Lateral bowing of the proximal femur is present at two levels (shepherd’s crook) as well as medial bowing of the distal femur. The neck-shaft angle is varus (100°) (reprinted from Ippolito et al. [21])

Heterogenous

fibrous dysplasia mimicking metastatic malignancy in patient with localised lung cancer. Initial FDG PET/CT, whole body attenuation corrected MIP, demonstrating mild uptake (thin yellow circle) with a small intense component (thick red arrow) in left femoral intertrochanteric region.

Heterogenous

fibrous dysplasia mimicking metastatic malignancy in patient with localised lung cancer. Fused axial images of the same FDG PET/CT in supine position demonstrating an indeterminate mildly active fibro-osseous lesion in the left femoral intertrochanteric region with an associated intensely hypermetabolic posterior nodule (red arrow).

Heterogenous

fibrous dysplasia mimicking metastatic malignancy in patient with localised lung cancer. T2FSE fat saturation axial MR depicts a hyperintense, non-fat containing lesion at the left femoral intertrochanteric region with posterior medial cortical thinning.

Heterogenous

fibrous dysplasia mimicking metastatic malignancy in patient with localised lung cancer. T1FSE pre-contrast axial MR demonstrating the concerning lesion (red arrow), measuring 1.7x1.6x1.4 cm, at the left femoral intertrochanteric region with posterior medial cortical thinning.

Heterogenous

fibrous dysplasia mimicking metastatic malignancy in patient with localised lung cancer. T1FSE fat saturation, post-contrast axial MR demonstrates avid enhancement of the same posterior heterogeneous lesion (red arrow).

Heterogenous

fibrous dysplasia mimicking metastatic malignancy in patient with localised lung cancer. Planning images for CT-guided biopsy (patient prone) depict the same fibro-osseous lesion with focal posterior nodule demonstrating cortical thinning. These homogeneously sclerotic, ground glass opacities of the femoral neck are classic for fibrous dysplasia.