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Histiocytic Syndromes

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Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Abstract

In addition to Langerhans cell histiocytosis and its variants, and histiocytic sarcoma, the hepatobiliary tract can be involved by a wide spectrum of other histiocytic neoplastic and nonneoplastic disorders. Rosai-Dorfman syndrome (sinus histiocytosis with massive lymphadenopathy), a polyclonal monocyte/macrophage/histiocyte disorder which involves various organs and tissue systems, can also present in the liver in the form of an atypical histiocytic infiltration, but hepatobiliary involvement is a rare event. Involvement of the liver, sometimes severe, is known for adult xanthogranulomatous disease, of which Erdheim-Chester disease is a prominent member. The liver frequently participates in various forms of hemophagocytic syndromes, including macrophage activation syndromes. Hemophagocytic lymphohistiocytosis is a heterogeneous group of inherited and acquired disorders of monocyte/macrophage function. The disorders include hereditary forms (familial hemophagocytic histiocytosis) and a broad array of histiocyte/macrophage disorders associated with or induced by autoimmune processes, collagen vascular diseases, tumors, and infections. In the liver, these disorders produce important macrophage accumulation associated with hemophagocytosis and sometimes destruction of small bile ducts, similar to biliary autoimmune diseases. A reactive histiocytosis associated with immunoglobulin-producing lymphomas is crystal-storing histiocytosis.

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  1. University of Bern, Bern, Switzerland

    Arthur Zimmermann

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Zimmermann, A. (2017). Histiocytic Syndromes. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_103

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  • DOI: https://doi.org/10.1007/978-3-319-26956-6_103

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