TITLE

Biliary Manifestation of Erdheim–Chester Disease Mimicking Klatskin's Carcinoma

AUTHOR(S)
Gundling, Felix; Nerlich, Andreas; Heitland, Wolf-Ulrich; Schepp, Wolfgang
PUB. DATE
February 2007
SOURCE
American Journal of Gastroenterology;Feb2007, Vol. 102 Issue 2, p452
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Erdheim–Chester disease (ECD) is a rare non-Langerhans form of histiocytosis, characterized by infiltration of foamy, lipid-laden histiocytes often affecting the lower extremities and resulting in symmetrical osteosclerosis. Internal organs involved include the lung and the kidneys, reflecting progressive disseminated granulomatous infiltration. Progression may be fatal, especially in cases of systemic extraosseous involvement. Here, we report a patient with elevated serum levels of liver enzymes due to intra- and extrahepatic bile duct stenoses. The patient's past medical history was remarkable for ECD, since 1 yr before he had undergone surgery for a pituitary lesion in our neurosurgical department revealing the typical histological and immunohistochemical criteria of ECD. Because no biliary manifestation of ECD had been described so far in the literature, surgery of suspected bile duct carcinoma was performed unraveling an unresectable tumor of the hilar region. Surprisingly, histologic examination of intraoperative biopsy specimens failed to demonstrate malignancy but rather revealed another xanthogranulomatous lesion embedded in extended periductal fibrosis as is typically described in extrahepatic parenchymal organ manifestation of ECD. Other possible reasons for cholestatic liver disease were excluded. Secondary cholestasis was overcome by endoscopic dilatation and biliary stenting with stents being exchanged every 3 months. During follow-up for 7 yr we have observed only a slight increase of the hilar stenosis so far. This is the first report describing biliary manifestation of ECD. Even though ECD is a rare cause of cholestasis, it should be considered in patients with this disorder in the setting of multiorgan manifestation.
ACCESSION #
23774310

 

Related Articles

  • Langerhans Cell Histiocytosis and Choledocholithiasis: Is There an Association? Naveneethan, Udayakumar; Parsi, Mansour A. // Gastroenterology & Hepatology;Nov2012, Vol. 8 Issue 11, p768 

    The article ponders on the results of a case study on the association between Langerhans cell histiocytosis (LCH) and choledocholithiasis. LCH is known as a rare disease associated with clonal proliferation of pathologic cells. Patients with LCH suffers organ dysfunction due to the infiltration...

  • Cure needed for melorheostosis. P.G.O'C. // Cortlandt Forum;10/25/97, Vol. 10 Issue 10, p141 

    Provides information on melorheostosis, a form of osteosclerosis or hyperostosis typically found on one of the long bones of the extremities. Presence of a linear proliferation of new bone; Limit of range of motion that results in disability and dysfunction.

  • Incidence, Diagnosis, and Therapy of Cholangiocarcinoma in Patients with Primary Sclerosing Cholangitis. Johan Fevery; Chris Verslype; Gillian Lai; Raymond Aerts; Werner Van Steenbergen // Digestive Diseases & Sciences;Nov2007, Vol. 52 Issue 11, p3123 

    Abstract  Primary sclerosing cholangitis (PSC) can lead to the development of cholangiocarcinoma (CCA). The tumor may present as an intrahepatic focal cholangiocellular carcinoma but more often as a ductal infiltrating desmoplastic lesion. CCA is found synchronously with the...

  • A Case of Erdheim-Chester Disease with Asymptomatic Renal Involvement. Hyun Jung Lee; Kyoung Yul Lee; Dong-Yeop Shin; Yun Gyoo Lee; Se Youn Choi; Kyung Chul Moon; Il-Kyu Han; Tae Min Kim // Cancer Research & Treatment;Jun2012, Vol. 44 Issue 2, p146 

    Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis involving bones and multiple organs. Its clinical course can vary, from an asymptomatic state to a fatal disease, with renal involvement being a common cause of death. A 41-year-old man presented with a 10-month history of...

  • Palliation of Nonresectable Bile Duct Cancer: Improved Survival After Photodynamic Therapy. Zoepf, Thomas; Jakobs, Ralf; Arnold, Joachim C.; Apel, Darius; Riemann, Jürgen F. // American Journal of Gastroenterology;Nov2005, Vol. 100 Issue 11, p2426 

    OBJECTIVES: Preliminary uncontrolled studies of photodynamic therapy (PDT) of bile duct cancer (BDC) have shown astonishingly good results in the reduction of cholestasis, improvement of life quality, and potential improvement of survival time. Therefore, we investigated the influence of PDT on...

  • Radiofrequency ablation as a treatment for hilar cholangiocarcinoma. Wei-Jun Fan; Pei-Hong Wu; Liang Zhang; Jin-Hua Huang; Fu-Jun Zhang; Yang-Kui Gu; Ming Zhao; Xiang-Long Huang; Chang-Yu Guo; Arai, Masahiro; Villa-Trevio, Saúl // World Journal of Gastroenterology;7/28/2008, Vol. 14 Issue 28, p4540 

    AIM: To explore the role of radio-frequency ablation (RFA) as a treatment for hilar cholangiocarcinoma. METHODS: Eleven patients with obstructive cholestasis underwent Computed Tomography (CT) examination, occupying lesions were observed in the hepatic hilar region in each patient. All lesions...

  • Cholangiocarcinoma presenting in an adolescent with vertically acquired HIV infection. Mangeya, N.; Mafukidze, A. T.; Pascoe, M.; Mbuwayesango, B.; Madziva, D.; Ndlovu, N.; Corbett, E. L.; Miller, R. F.; Ferrand, R. A. // International Journal of STD & AIDS;Oct2008, Vol. 19 Issue 10, p717 

    An adolescent with long-standing HIV infection who was severely immunosuppressed during HIV diagnosis developed cholangiocarcinoma 1.5 years after starting antiretroviral therapy.

  • A Gentleman with Anemia and Cholestasis. Siu-Tong Law; Wai-Ki Lee; Michael Kin-Kong Li; Ka-Ho Lok // Case Reports in Medicine;2010, p1 

    Primary sclerosing cholangitis is a rare cause of cholestasis caused by progressive inflammation and fibrosis of both intrahepatic and extrahepatic bile ducts leading to multifocal ductal strictures. Herein, we report a case of primary sclerosing cholangitis and inflammatory bowel disease. The...

  • Research & Markets: Bile Duct Cancer Global Clinical Trials Review, H2 2011.  // Biomedical Market Newsletter;1/7/2012, Vol. 21, p1 

    The article presents information on the new report of GlobalData, "Bile Duct Cancer Global Clinical Trials Review, H2 2011". It is mentioned that the report gives the overview on the Bile Duct Cancer clinical Trial scenario and provides elemental information on the trial. The report further...

Share

Read the Article

Courtesy of THE LIBRARY OF VIRGINIA

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics