EURORAD ESR

Case 11919

Erdheim–Chester disease (ECR 2014 Case of the Day)

Author(s)
R. Colantonio1, A.R. Larici1, T. Verdolotti1, A. Meduri1, G. Petrone2, A. Mulè2, C. Colosimo1, L. Bonomo1

1Department of Bioimaging and Radiological Sciences,
Catholic University of Rome,
Agostino Gemelli Hospital; Rome/IT
2Department of Pathology,
Catholic University of Rome,
Agostino Gemelli Hospital; Rome/IT

Email:raffaella.colantonio@gmail.com
 
Patient
female, 44 year(s)
 
 
  • Figure 1
    Head CT

    Preoperative unenhanced head CT (1a) showing bilateral homogeneous retrobulbar tissue. Postoperative unenhanced head CT after surgical orbital decompression (1b). Please note the absence of the lateral and medial...

     
    Area of Interest: Head and neck; Imaging Technique: CT; Procedure: Education; Special Focus: Pathology;
     
     
  • Figure 2
    Brain MRI

    Brain MRI showing bilateral intraconal homogeneous retrobulbar tissue surrounding optic nerves, hypointense on T2WI (2a), with mild homogeneous enhancement on contrast-enhanced T1WI (2b).

     
    Area of Interest: Head and neck; Imaging Technique: MR; Procedure: Education; Special Focus: Pathology;
     
     
  • Figure 3
    Chest radiograph

    Chest radiograph showing diffuse reticular interstitial involvement, fissural thickening and enlarged cardiac image.

     
    Area of Interest: Thorax; Imaging Technique: Conventional radiography; Procedure: Education; Special Focus: Pathology;
     
     
  • Figure 4
    Chest CT

    Axial images at lung window setting (4a, b) showing interlobular thickening, fissural thickening, pleuropericardial effusion. Axial images at mediastinal window setting (4c) showing tissue infiltrating right atrium...

     
    Area of Interest: Thorax; Imaging Technique: CT; Procedure: Education; Special Focus: Pathology;
     
     
  • Figure 5
    Cardiac MRI

    Cardiac-gated T2WI showing pericardial effusion and low-signal tissue surrounding circumferentially right coronary artery (yellow arrow) and infiltrating right atrial wall (5a). Late-enhancement cardiac MR showing...

     
    Area of Interest: Cardiac; Imaging Technique: MR; Procedure: Education; Special Focus: Pathology;
     
     
  • Figure 6
    Abdominal CT

    Enhanced abdominal CT showing bilateral perirenal infiltration extending into the renal sinus of kidneys. Please note also circumferential periaortic infiltration.

     
    Area of Interest: Abdomen; Imaging Technique: CT; Procedure: Education; Special Focus: Pathology;
     
     
  • Figure 7
    Percutaneous pyelography

    Percutaneous pyelography showing marked dilatation of the right renal collecting system due to the stenosis of the upper portion of ureter (yellow arrow).

     
    Area of Interest: Kidney; Imaging Technique: Percutaneous; Procedure: Pyelography; Special Focus: Pathology;
     
     
  • Figure 8
    Radiograph of distal femurs

    Radiograph of distal femurs showing bilateral and symmetric heterogeneous osteosclerosis of diaphyseal and metaphyseal regions.

     
    Area of Interest: Bones; Imaging Technique: Plain radiographic studies; Procedure: Education; Special Focus: Pathology;
     
     
  • Figure 9
    Histological analysis

    Etmoidal mucosa showing a chronic inflammatory infiltrate rich in macrophages sometimes with foamy cytoplasm. (Haematoxylin and Eosin; original magnification x100) (9a). Macrophages exhibiting cytoplasmatic...

     
    Area of Interest: Education; Imaging Technique: Experimental; Procedure: Education; Special Focus: Pathology;
     
     
Preoperative unenhanced head CT (1a) showing bilateral homogeneous retrobulbar tissue. Postoperative unenhanced head CT after surgical orbital decompression (1b). Please note the absence of the lateral and medial orbital wall (yellow arrows).
 
Brain MRI showing bilateral intraconal homogeneous retrobulbar tissue surrounding optic nerves, hypointense on T2WI (2a), with mild homogeneous enhancement on contrast-enhanced T1WI (2b).
 
Chest radiograph showing diffuse reticular interstitial involvement, fissural thickening and enlarged cardiac image.
 
Axial images at lung window setting (4a, b) showing interlobular thickening, fissural thickening, pleuropericardial effusion. Axial images at mediastinal window setting (4c) showing tissue infiltrating right atrium and surrounding right coronary artery (yellow arrow).
 
Cardiac-gated T2WI showing pericardial effusion and low-signal tissue surrounding circumferentially right coronary artery (yellow arrow) and infiltrating right atrial wall (5a). Late-enhancement cardiac MR showing strong enhancement of the tissue and thickened pericardium (5b).
 
Enhanced abdominal CT showing bilateral perirenal infiltration extending into the renal sinus of kidneys. Please note also circumferential periaortic infiltration.
 
Percutaneous pyelography showing marked dilatation of the right renal collecting system due to the stenosis of the upper portion of ureter (yellow arrow).
 
Radiograph of distal femurs showing bilateral and symmetric heterogeneous osteosclerosis of diaphyseal and metaphyseal regions.
 
Etmoidal mucosa showing a chronic inflammatory infiltrate rich in macrophages sometimes with foamy cytoplasm. (Haematoxylin and Eosin; original magnification x100) (9a). Macrophages exhibiting cytoplasmatic immunoreactivity for CD68 (9B), PGM-1 (9C) (Haematoxylin counterstaining; original magnification x100).
 
 
 
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