EURORAD ESR

Case 14620

Erdheim Chester disease - Brain: Typical MRI findings

Author(s)
J.A Prat-Matifoll; L.Causil Garcia; B. del Rio; R. Mitjana Penella

Vall Hebron Hospital,
Institut Català de la Salut,
Radiology;
Passeig Vall Hebrón 116-119
08035 Barcelona, Spain;
Email:joanalbertpratrx@gmail.com
 
Patient
male, 41 year(s)
 
 
  • Figure 1
    Brain MRI: Findings
     

    On T1-WI, the lesion exhibits a well-defined anterior outline (white arrow) and predominantly diffuse hyperintense signal. Note the hyperintense thickening of tentorium cerebelli (red arrows).

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Neoplasia;

    Large non-parenchymal mass, well defined and centered in the posterior cerebral falx. Its signal is markedly hypointense on T2-WI (white arrows).

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Neoplasia;

    This lesion causes a moderate mass effect on the splenium of corpus callosum, observing a hyperintense signal on FLAIR sequences, probably due to the presence of vasogenic oedema (red arrows).

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Neoplasia;

    Coronal T2WI (left) and T1WI (right) of the orbits. Hypointense masses on T1/T2 engulfing the right optical nerve (red arrows) and on the upper left side of the left globe (yellow arrows).

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Inflammation;

    Axial T2WI (left images) and T1WI (right images) showing the typical hypointensity on both sequences (red and yellow arrows).

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Tissue characterisation;
     
     
  • Figure 2
    Diffusion (DWI) and Susceptibility-weighted imaging (SWI)

    There is neither hyperintesity on B-1000 (left image) nor hypointensity on ADC (not significantly low ADC values on the right image) suggesting diffusion restriction.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Neoplasia;
     
     
  • Figure 3
    Contrast-enhanced MRI
     

    Meningioma-like dural mass extending bilaterally to the tentorium cerebelli and showing a homogeneous and intense enhancement on T1WI.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Neoplasia;

    Note how the dural mass invades the straight sinus (red arrows) and the Vein of Galen.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Neoplasia;

    Dilated bilateral Labbe veins (red arrows) to compensate the lack of venous drainage through the straight sinus.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Neoplasia;

    Two orbital lesions were detected after contrast administration (yellow and red arrows). Note the presence of 2 intraconal soft-tissue lesions showing intense enhancement on T1WI (red arrows) and causing a slight...

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Neoplasia;
     
     
  • Figure 4
    Musculoskeletal findings: Plain films
     

    Osseous involvement, which appears as medullary long bone osteosclerosis in the metaphyseal and diaphyseal region. Note the epiphyseal sparing.

     
    Area of Interest: Musculoskeletal system; Imaging Technique: Plain radiographic studies; Procedure: Education; Special Focus: Neoplasia;

    Sclerotic coarsened trabeculae with regions of lysis in the metaphyses/diaphyses of femoral and tibial bones.

     
    Area of Interest: Musculoskeletal system; Imaging Technique: Plain radiographic studies; Procedure: Education; Special Focus: Neoplasia;

    Note the characteristic patchy medullary osteosclerosis in the metaphyseal and diaphyseal region with epiphyseal sparing. Irregularity/waviness of the cortex may indicate periosteal involvement.

     
    Area of Interest: Musculoskeletal system; Imaging Technique: Plain radiographic studies; Procedure: Education; Special Focus: Neoplasia;

    Note that the affection is symmetric and remember, the epiphyses are spared.

     
    Area of Interest: Musculoskeletal system; Imaging Technique: Plain radiographic studies; Procedure: Education; Special Focus: Neoplasia;
     
     
  • Figure 5
    Whole body PET-CT: findings
     

    Characteristic perirenal rind of soft tissue (red arrows), usually asymptomatic but can cause impingement on the renal vasculature or collecting system.

     
    Area of Interest: Kidney; Imaging Technique: CT; Procedure: Education; Special Focus: Neoplasia;

    The perirenal rind of soft tissue typically shows an increased uptake of FDG (white and black arrows).

     
    Area of Interest: Kidney; Imaging Technique: PET-CT; Procedure: Education; Special Focus: Neoplasia;

    Perivascular rind of soft tissue around the descending aorta and the left subclavian artery (yellow arrows).

     
    Area of Interest: Vascular; Imaging Technique: CT-Angiography; Procedure: Education; Special Focus: Neoplasia;

    Elevated metabolic activity involving the aortic arch (white arrow, left subclavian artery) and the descending aorta (white arrow).

     
    Area of Interest: Vascular; Imaging Technique: PET; Procedure: Education; Special Focus: Neoplasia;

    Cortical thickening (endosteal thickening) with sclerotic coarsened trabeculae and regions of lysis in the metaphyses/diaphyses of femoral bones (blue arrows). Multiple vertebrae with sclerotic coarsened trabeculae...

     
    Area of Interest: Bones; Imaging Technique: CT; Procedure: Education; Special Focus: Neoplasia;
     
     
On T1-WI, the lesion exhibits a well-defined anterior outline (white arrow) and predominantly diffuse hyperintense signal. Note the hyperintense thickening of tentorium cerebelli (red arrows).
 
Large non-parenchymal mass, well defined and centered in the posterior cerebral falx. Its signal is markedly hypointense on T2-WI (white arrows).
 
This lesion causes a moderate mass effect on the splenium of corpus callosum, observing a hyperintense signal on FLAIR sequences, probably due to the presence of vasogenic oedema (red arrows).
 
Coronal T2WI (left) and T1WI (right) of the orbits. Hypointense masses on T1/T2 engulfing the right optical nerve (red arrows) and on the upper left side of the left globe (yellow arrows).
 
Axial T2WI (left images) and T1WI (right images) showing the typical hypointensity on both sequences (red and yellow arrows).
 
There is neither hyperintesity on B-1000 (left image) nor hypointensity on ADC (not significantly low ADC values on the right image) suggesting diffusion restriction.
 
Meningioma-like dural mass extending bilaterally to the tentorium cerebelli and showing a homogeneous and intense enhancement on T1WI.
 
Note how the dural mass invades the straight sinus (red arrows) and the Vein of Galen.
 
Dilated bilateral Labbe veins (red arrows) to compensate the lack of venous drainage through the straight sinus.
 
Two orbital lesions were detected after contrast administration (yellow and red arrows). Note the presence of 2 intraconal soft-tissue lesions showing intense enhancement on T1WI (red arrows) and causing a slight bilateral exophthalmos.
 
Osseous involvement, which appears as medullary long bone osteosclerosis in the metaphyseal and diaphyseal region. Note the epiphyseal sparing.
 
Sclerotic coarsened trabeculae with regions of lysis in the metaphyses/diaphyses of femoral and tibial bones.
 
Note the characteristic patchy medullary osteosclerosis in the metaphyseal and diaphyseal region with epiphyseal sparing. Irregularity/waviness of the cortex may indicate periosteal involvement.
 
Note that the affection is symmetric and remember, the epiphyses are spared.
 
Characteristic perirenal rind of soft tissue (red arrows), usually asymptomatic but can cause impingement on the renal vasculature or collecting system.
 
The perirenal rind of soft tissue typically shows an increased uptake of FDG (white and black arrows).
 
Perivascular rind of soft tissue around the descending aorta and the left subclavian artery (yellow arrows).
 
Elevated metabolic activity involving the aortic arch (white arrow, left subclavian artery) and the descending aorta (white arrow).
 
Cortical thickening (endosteal thickening) with sclerotic coarsened trabeculae and regions of lysis in the metaphyses/diaphyses of femoral bones (blue arrows). Multiple vertebrae with sclerotic coarsened trabeculae (yellow arrows).
 
 
 
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