EURORAD ESR

Case 14740

Erdheim-Chester disease

Author(s)
Korving J, Festhali S

Leiden University Medical Centre,
Radiology;
Albinusdreef 2
2333 ZA Leiden, Netherlands;
Email:j.c.korving@lumc.nl
 
Patient
female, 46 year(s)
 
 
  • Figure 1
    Thick pituitary stalk
     

    Coronal MR image after gadolinium administration. A diffuse enhancing pituitary stalk mass.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Contrast agent-intravenous; Special Focus: Endocrine disorders;

    Sagittal MR image after gadolinium administration. A diffuse enhancing pituitary stalk mass.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Contrast agent-intravenous; Special Focus: Endocrine disorders;
     
     
  • Figure 2
    Pericardial effusion & coated aorta
     

    Coronal CT reconstruction. Pericardial effusion and 'coating' of the ascending aorta.

     
    Area of Interest: Cardiovascular system; Imaging Technique: CT; Procedure: Contrast agent-intravenous; Special Focus: Pathology;

    Axial CT with iv contrast. Soft tissue encasement of the aortic arch, the 'coated aorta' appearance.

     
    Area of Interest: Arteries / Aorta; Imaging Technique: CT; Procedure: Contrast agent-intravenous; Special Focus: Pathology;

    Axial CT with iv contrast. Soft tissue encasement of the aortic arch branches.

     
    Area of Interest: Vascular; Imaging Technique: CT; Procedure: Contrast agent-intravenous; Special Focus: Pathology;
     
     
  • Figure 3
    Hairy kidneys & enlargement of the adrenal glands.
     

    Coronal multiplanar reconstruction. Bilateral soft tissue encasement of the kidneys with bilateral dilatation of the renal pelvis. There is also bilateral infiltration and enlargement of the adrenal glands.

     
    Area of Interest: Kidney; Imaging Technique: CT; Procedure: Contrast agent-intravenous; Special Focus: Dilatation;

    Axial CT on the level of the kidneys showing bilateral soft tissue encasement of the kidneys with bilateral dilatation of the renal pelvis.

     
    Area of Interest: Kidney; Imaging Technique: CT; Procedure: Contrast agent-intravenous; Special Focus: Dilatation;

    Axial CT on the level of mainly the right adrenal showing infiltration and enlargement of the adrenal glands.

     
    Area of Interest: Adrenals; Imaging Technique: CT; Procedure: Contrast agent-intravenous; Special Focus: Pathology;
     
     
  • Figure 4
    Osteosclerosis of the distal femur diaphysis
     

    X-rays of the distal femora showing bilateral patchy sclerosis of the distal femur diaphysis and proximal left tibia.

     
    Area of Interest: Extremities; Imaging Technique: Conventional radiography; Procedure: Sclerosis; Special Focus: Pathology;

    Bone scintigraphy. Besides bilateral increased uptake in the distal femur and proximal left tibia, there is also slight increased uptake in the proximal right tibia, bilateral in the proximal humerus and mandible.

     
    Area of Interest: Bones; Imaging Technique: Absorptiometry / Bone densiometry; Procedure: Ablation procedures; Special Focus: Pathology;
     
     
  • Figure 5
    Bilateral retro-orbital masses
     

    MR T1w STIR after gadolinium injection shows bilateral enhancing retro-orbital masses.

     
    Area of Interest: Eyes; Imaging Technique: MR; Procedure: Contrast agent-intravenous; Special Focus: Pathology;

    Coronal MR T1 STIR after gadolinium injection shows bilateral enhancing retro-orbital masses.

     
    Area of Interest: Eyes; Imaging Technique: MR; Procedure: Contrast agent-intravenous; Special Focus: Pathology;
     
     
  • Figure 6
    Focal thickening of the right atrium
     

    Axial cardiac-gated T2 weighted imaging showing pericardial effusion and slight thickening of the free wall of the right atrium.

     
    Area of Interest: Cardiac; Imaging Technique: MR; Procedure: Education; Special Focus: Pathology;

    Late-enhancement cardiac MR showing enhancement of the thickened free wall of the right atrium.

     
    Area of Interest: Cardiac; Imaging Technique: MR; Procedure: Education; Special Focus: Pathology;
     
     
Coronal MR image after gadolinium administration. A diffuse enhancing pituitary stalk mass.
 
Sagittal MR image after gadolinium administration. A diffuse enhancing pituitary stalk mass.
 
Coronal CT reconstruction. Pericardial effusion and 'coating' of the ascending aorta.
 
Axial CT with iv contrast. Soft tissue encasement of the aortic arch, the 'coated aorta' appearance.
 
Axial CT with iv contrast. Soft tissue encasement of the aortic arch branches.
 
Coronal multiplanar reconstruction. Bilateral soft tissue encasement of the kidneys with bilateral dilatation of the renal pelvis. There is also bilateral infiltration and enlargement of the adrenal glands.
 
Axial CT on the level of the kidneys showing bilateral soft tissue encasement of the kidneys with bilateral dilatation of the renal pelvis.
 
Axial CT on the level of mainly the right adrenal showing infiltration and enlargement of the adrenal glands.
 
X-rays of the distal femora showing bilateral patchy sclerosis of the distal femur diaphysis and proximal left tibia.
 
Bone scintigraphy. Besides bilateral increased uptake in the distal femur and proximal left tibia, there is also slight increased uptake in the proximal right tibia, bilateral in the proximal humerus and mandible.
 
MR T1w STIR after gadolinium injection shows bilateral enhancing retro-orbital masses.
 
Coronal MR T1 STIR after gadolinium injection shows bilateral enhancing retro-orbital masses.
 
Axial cardiac-gated T2 weighted imaging showing pericardial effusion and slight thickening of the free wall of the right atrium.
 
Late-enhancement cardiac MR showing enhancement of the thickened free wall of the right atrium.
 
 
 
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