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Tumors of the Langerhans Cell System

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Abstract

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of bone marrow-derived antigen-presenting cells that home to diverse organs and tissues, predominantly the skin. LCH is a rare disease that mainly manifests in the pediatric age group, with several in part overlapping phenotypes. Clinically and morphologically, LCH is divided into unifocal LCH, single-system LCH, multifocal LCH, multiorgan/disseminated LCH, Langerhans cell sarcoma, and Langerhans tumors. All forms and variants are characterized by a progressive and monoclonal proliferation of medium-sized, CD1a-reactive, and langerin-/CD207-reactive cells that often contain Birbeck granules. Langerhans cell infiltrates are regularly accompanied by infiltrates of eosinophils, lymphoid cells, and macrophages. Lesions with a high density of eosinophils were previously termed eosinophilic granuloma. Hepatobiliary involvement in LCH presents with a characteristic spectrum of lesions that include a disseminated form, focal forms (microscopic and macroscopic), and a form associated with bile ducts (LCH cholangiopathy). A small subset of Langerhans cell proliferations present as tumorous lesions or Langerhans cell sarcoma. In addition to LCH, there are a group of so-called self-healing Langerhans cell and related disorders that can involve the hepatobiliary tract.

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Zimmermann, A. (2017). Tumors of the Langerhans Cell System. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_100

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