EURORAD ESR

Case 14798

An unusual infiltrative disease of brain: the Erdheim-Chester disease

Author(s)
Giuseppe Buragina 1, Soldi Simone 1, Anna Maria Ierardi1, Elena Lovati1, Giovanni Pompili1, Sergio Serantoni1, Gian Marco Podda2, Enrico Maria Fumarola1, Alberto Magenta Biasina1, Gianpaolo Carrafiello1

1 Department of Radiology, San Paolo Hospital, Milan, Italy
2 Department of Medicine San Paolo Hospital, Milan, Italy
 
Patient
male, 43 year(s)
 
 
  • Figure 1
    CT of orbital and pontine involvement
     

    Pre-contrast axial CT image shows bilateral retrobulbar pseudotumors with intraconal extension (red arrow) The optic nerves and extrinsic muscles are delocalized by the masses.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;

    The post contrast images show an homogenous enhancement of the retrobulbar masses (orange arrows). Note also the inhomogeneous enhancement in the pontine region due to histiocyte infiltration (red arrow).

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;
     
     
  • Figure 2
    MRI of brain
     

    The T2 FLAIR image shows the presence of hyperintense lesions in the hippocampus and parahippocampal gyri (black arrow). The disease infiltrates also the hypothalamic region (red arrow).

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;

    The sagittal T2 FLAIR describes the involvement of hypothalamus and tuber cinereum (red arrow).This findings may explain the central diabetes insipidus seen in our patient. Note also the pontine lesions.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;

    The DWI showed a mild restriction of diffusion of the pontine lesions (black arrow).

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;

    The corresponding ADC map does not show the presence of restricted diffusivity in the pons (blac arrow).

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;

    This axial post contrast T1 fast spin echo image reveals multiple, nodular enhancing lesions in the hippocampal ( orange arrow) and pontine region.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;
     
     
  • Figure 3
    Orbital involvement on MRI images
     

    The axial T1 image shows the presence of bilateral hypointense pseudotumoral masses in the retrobulbar space, which replace the normal fat (red arrows).

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;

    After the admnistration of gadolinium, the masses in the retrobulbar space show an homogenous enhancement (red arrow).

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;
     
     
  • Figure 4
    Cardiovascular, lung abdominal and bone involvement
     

    The contrast enhanced CT of thorax demonstrates the presence of a fibrotic mass in the rigth periatrial fat (orange arrow).

     
    Area of Interest: Cardiovascular system; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;

    An axial CT image of lungs shows the presence of thickned interlobular septa and ground glass areas in the upper lobes.

     
    Area of Interest: Lung; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;

    A wide fibrotic mass in the retroperitoneum (orange arrow) with perirenal and periaortic involvement, which produces the hairy kidneys sign (red arrow) and the coated aorta sign (black arrow),respectively.

     
    Area of Interest: Gastrointestinal tract; Imaging Technique: CT; Procedure: Defecography; Special Focus: Haematologic diseases;

    The technetium-99m bone scintigraphy describes a pathological uptake in the rigth distal femur (red arrow).

     
    Area of Interest: Bones; Imaging Technique: Nuclear medicine conventional; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;
     
     
Pre-contrast axial CT image shows bilateral retrobulbar pseudotumors with intraconal extension (red arrow) The optic nerves and extrinsic muscles are delocalized by the masses.
 
The post contrast images show an homogenous enhancement of the retrobulbar masses (orange arrows). Note also the inhomogeneous enhancement in the pontine region due to histiocyte infiltration (red arrow).
 
The T2 FLAIR image shows the presence of hyperintense lesions in the hippocampus and parahippocampal gyri (black arrow). The disease infiltrates also the hypothalamic region (red arrow).
 
The sagittal T2 FLAIR describes the involvement of hypothalamus and tuber cinereum (red arrow).This findings may explain the central diabetes insipidus seen in our patient. Note also the pontine lesions.
 
The DWI showed a mild restriction of diffusion of the pontine lesions (black arrow).
 
The corresponding ADC map does not show the presence of restricted diffusivity in the pons (blac arrow).
 
This axial post contrast T1 fast spin echo image reveals multiple, nodular enhancing lesions in the hippocampal ( orange arrow) and pontine region.
 
The axial T1 image shows the presence of bilateral hypointense pseudotumoral masses in the retrobulbar space, which replace the normal fat (red arrows).
 
After the admnistration of gadolinium, the masses in the retrobulbar space show an homogenous enhancement (red arrow).
 
The contrast enhanced CT of thorax demonstrates the presence of a fibrotic mass in the rigth periatrial fat (orange arrow).
 
An axial CT image of lungs shows the presence of thickned interlobular septa and ground glass areas in the upper lobes.
 
A wide fibrotic mass in the retroperitoneum (orange arrow) with perirenal and periaortic involvement, which produces the hairy kidneys sign (red arrow) and the coated aorta sign (black arrow),respectively.
 
The technetium-99m bone scintigraphy describes a pathological uptake in the rigth distal femur (red arrow).
 
 
 
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