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

MedStar author(s):
Citation: Balkan Medical Journal. 35(6):437-439, 2018 11 15.PMID: 29514770Institution: MedStar Washington Hospital CenterDepartment: PathologyForm of publication: Journal ArticleMedline article type(s): Journal ArticleSubject headings: *Bile Ducts, Extrahepatic/ab [Abnormalities] | *Histiocytosis, Langerhans-Cell/co [Complications] | Bile Ducts, Extrahepatic/pp [Physiopathology] | Female | Histiocytosis, Langerhans-Cell/di [Diagnosis] | Histiocytosis, Langerhans-Cell/pa [Pathology] | Humans | Middle Aged | Tomography, X-Ray Computed/mt [Methods]Year: 2018ISSN:
  • 2146-3123
Name of journal: Balkan medical journalAbstract: 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.All authors: Kallakury B, Obiorah IE, Ozdemirli M, Velasquez AHFiscal year: FY2019Fiscal year of original publication: FY2018Digital Object Identifier: Date added to catalog: 2018-04-20
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Journal Article MedStar Authors Catalog Article 29514770 Available 29514770

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.

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