Hepatic composite tumor in a patient with primary sclerosing cholangitis

A focal intrahepatic enhancing lesion with associated bile duct stricture was identified in a 25-year-old woman with a history of primary sclerosing cholangitis (PSC) who was being evaluated for increasing liver enzymes. Partial hepatectomy was performed, revealing a composite tumor with neuroendocrine and cholangiocarcinomatous differentiation. Only one composite tumor of the liver has been previously reported in the literature [H. Hidaka, S. Kuwao, S. Kokubu, K. Yanagimoto, A. Satomichi, M. Takada, T. Nakazawa, K. Saigenji, Mixed carcinoid-adenocarcinoma of the liver, Intern. Med. 39 (2000) 910-913], and to our knowledge, this is the first report of such a tumor in association with PSC. We report this very unusual case and discuss the potential association of neuroendocrine carcinoma with primary sclerosing cholangitis.