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Liver and intrahepatic bile ducts - tumor

Other malignancies

Erdheim-Chester disease


Reviewers: Deepali Jain, M.D. (see Reviewers page)
Revised: 9 January 2013, last major update February 2012
Copyright: (c) 2004-2013, PathologyOutlines.com, Inc.

General
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● 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

Treatment
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● Oral steroids, chemotherapy or radiotherapy in severe cases

Case reports
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● 32 year old man with vertebral destruction and liver involvement (Arch Pathol Lab Med 2003;127:e337)
● Biliary manifestation of Erdheim-Chester disease mimicking Klatskin's carcinoma (Am J Gastroenterol 2007;102:452)

Micro description
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● Diffuse infiltration of large, foamy histiocytes, lymphocytic aggregates and fibrosis
● Rare Touton-like giant cells

Micro images
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CT scan, H&E, CD68 and EM




Various images


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

Positive stains
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● CD68

Negative stains
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● CD1a, S100

Electron microscopy description
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● No Birbeck granules

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


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