Granular Cell Tumor at the Hepatic Duct Confluence Mimicking Klatskin Tumor: A Report of Two Cases and a Review of the Literature

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Background:Granular cell tumors are rare tumors most often located in the oral cavity, skin or subcutaneous tissue. The occurrence of this tumor in the biliary tree is extremely rare.Methods:Two patients are described presenting with biliary obstruction due to a tumor at the hepatic duct confluence. One patient is a 38-year-old white male with concomitant cutaneous granular cell tumors, and the other a 50-year-old white female.Results:Hilar excision was performed in both patients. Histopathology of the tumors revealed a proliferation of cells with granular cytoplasm, diagnosed as granular cell tumor.Conclusion:At preoperative examination, hilar granular cell tumors are difficult to differentiate from cholangiocarcinoma, sclerosing cholangitis or more common benign biliary tumors. Treatment consists of surgical excision after which prognosis is favorable.

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