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Liver and intrahepatic bile ducts - tumor

Other malignancies

Langerhans cell histiocytosis (LCH)

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

See also Lymph nodes-not lymphoma chapter


● Neoplastic disorder of Langerhans cells, formerly called histiocytosis X
● Causes 15% of sclerosing cholangitis in children, usually affects intrahepatic bile ducts
● Remarkable selectivity for bile ducts, eventually causes biliary cirrhosis
● May only affect liver; also affects lymph nodes, skin
● Laboratory: Gamma glutamyl transpeptidase is sensitive indicator of liver infiltration in children with LCH

Case reports

● 65 year old woman with sclerosing cholangitis 4 years after diagnosis of Langerhans cell histiocytosis (Hepatol Int 2010;4:653)

Micro description

● Langerhans cells may surround bile ducts and infiltrate sinusoids in nodules or diffusely
● Associated with cirrhosis, sclerosing cholangitis, eosinophils, lymphocytes, plasma cells and neutrophils (Mod Pathol 1999;12:370)

Micro images

Langerhans cell histiocytosis - site unspecified

Sclerosing cholangitis caused by LCH, but no LCH involvement of liver itself

Positive stains

● S100, CD1a

End of Liver and intrahepatic bile ducts - tumor > Other malignancies > Langerhans cell histiocytosis

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