Liver and intrahepatic bile ducts - tumor
Other malignancies
Erdheim-Chester disease

Topic Completed: 1 February 2012

Revised: 28 January 2019, last major update February 2012

Copyright: (c) 2002-2017,, Inc.

PubMed Search: Erdheim-Chester disease liver

Deepali Jain, M.D.
Page views in 2019: 208
Page views in 2020 to date: 58
Cite this page: Jain D. Erdheim-Chester disease. website. Accessed April 1st, 2020.
Definition / general
  • Very rare disorder (< 100 cases described) of unknown etiology with systemic xanthogranulomatous histiocytosis
  • Usually adults, ages 30 - 70 years
  • Usually symmetric osteosclerosis of long bones and axial skeleton involvement
  • 50% have extraskeletal lesions of retroperitoneum, lung, kidney, brain, heart or retro-orbital space
  • Death in 60% due to respiratory and heart failure
Case reports
  • Oral steroids, chemotherapy or radiotherapy in severe cases
Microscopic (histologic) description
  • Diffuse infiltration of large, foamy histiocytes, lymphocytic aggregates and fibrosis
  • Rare Touton-like giant cells
Microscopic (histologic) images

Images hosted on other servers:

CT scan, H&E, CD68 and EM

Various images

Macrophages in (A)
testis, (B) thyroid,
(C) lymph node,
(D) testis: CD68

Positive stains
Negative stains
Electron microscopy description
  • No Birbeck granules
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