Ifeyinwa E. OBIORAH, Metin ÖZDEMİRLİ, Alicia Henao VELASQUEZ, Bhaskar KALLAKURY

Primary Langerhans Cell Histiocytosis of the Extrahepatic Bile Duct Occurring in an Adult Patient

Background: Langerhans cell histiocytosis is characterized by anabnormal proliferation of neoplastic Langerhans cells. Langerhanscell histiocytosis commonly affects the pediatric population, whereaspresentation in adults remains a rare event. The presentation ofLangerhans cell histiocytosis is highly variable, but the involvementof skin, bone, and lung is very common. Langerhans cell histiocytosispresenting as a bile duct mass is rare and usually occurs as part of amultiorgan system disease.Case Report: We present a case of Langerhans cell histiocytosisconfined to the extrahepatic bile duct in a 62-year-old female patientwith sclerosing cholangitis. The mass was composed of mononuclearcells with cleaved nuclei that were positive for CD68, S100, andCD1a as assessed by immunohistochemistry.Conclusion: This is the first report of Langerhans cell histiocytosislimited to the extrahepatic bile duct in an adult patient. We discussthe clinical manifestations and the challenges encountered in thediagnosis and treatment of this rare entity.

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