Erdheim-Chester disease

Case contributed by Dr Michael P Hartung

Presentation

Urinary obstruction of unknown cause.

Patient Data

Age: 55 years
Gender: Male
CT

Presentation

Ill-defined, strandy soft tissue in the renal hila and proximal ureters, resulting in mild bilateral hydronephrosis. This is fairly well seen on the coronal reformatted MIP images. 

Soft tissue thickening surrounding portions of the left kidney. 

Subtle short-segment circumferential thickening/enhancement of the mid-abdominal aorta. 

CT

3 year follow-up

Similar appearance of mild bilateral hydronephrosis and soft tissue thickening/stranding in the renal hila/proximal ureters. Soft tissue thickening around the right kidney. 

Similar short segment circumferential thickening surrounding the mid abdominal aorta. 

Possibly early mild thickening surrounding the aortic arch. 

Notable negatives: Normal adrenals and RCA.

CT

5 year follow-up

Increased circumferential soft tissue thickening or "coating" of the thoracic aorta and proximal great vessels. 

Soft tissue surrounding the RCA and filling the right atrioventricular groove.

Partially imaged infiltration of the adrenals. 

CT

9 year follow-up

Soft tissue infiltration of the adrenals, right greater than left.  

Thickening of the gallbladder and cystic duct. 

Coating of the abdominal aorta and proximal common iliac. 

Narrowing of the IVC with soft tissue infiltration.

Severely hydronephrotic right kidney with stent. 

Mild hydronephrosis left kidney. Perinephric and perihilar soft tissue infiltration ("hairy kidney"). 

Case Discussion

Rare case showing a relatively gradual progression of findings typical for Erdheim-Chester disease. The patient presented with renal obstruction due to ill-defined, infiltrative soft tissue in the renal hila and proximal ureters. 

Over the course of 9 years, the patient developed several more manifestations of the disease:

  • increasing renal involvement, resulting in obstruction of the right kidney and typical "hairy kidney" appearance of the left
  • adrenal infiltration
  • biliary involvement with gallbladder and cystic duct thickening
  • coating of the proximal great vessels and thoracic and abdominal aorta
  • involvement and narrowing of the IVC
  • infiltration of the RCA and right AV groove

Please see companion case with many overlapping imaging features. 

PlayAdd to Share

Case information

rID: 70576
Published: 30th Aug 2019
Last edited: 2nd Sep 2019
Inclusion in quiz mode: Included

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.