ISSN : 2146-3123
E-ISSN : 2146-3131

Primary Langerhans Cell Histiocytosis of the Extrahepatic Bile Duct Occurring in an Adult Patient
Ifeyinwa E. Obiorah1, Alicia Henao Velasquez1, Bhaskar Kallakury1, Metin Özdemirli1
1Clinic of Pathology, Medstar Georgetown University Hospital, Washington, USA
DOI : 10.4274/balkanmedj.2017.1730

Background: Langerhans cell histiocytosis is characterized by abnormal proliferation of neoplastic Langerhans cells. Langerhans cell histiocytosis commonly affects the pediatric population and presentation in adults remain a rare event. The presentation of langerhans cell histiocytosis is highly variable but skin, bone and lung involvement are very common. Langerhans cell histiocytosis presenting as a bile duct mass is rare and usually occurs as part of a multi-organ system disease.
Case Report: We present a case of langerhans cell histiocytosis confined to the extrahepatic bile duct in a 62-year-old female with sclerosing cholangitis. The mass was composed of mononuclear cells with cleaved nuclei that were positive for CD68, S100 and CD1a by immunohistochemistry.
Conclusion: This is the first report of langerhans cell histiocytosis limited to the extrahepatic bile duct in an adult patient. We discuss the clinical manifestations and challenges encountered in the diagnosis and treatment of this rare entity

Keywords : Langerhans cell, histiocytosis, bile duct, sclerosing cholangitis

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