Spindle cell carcinoma of the intrahepatic bile duct in a patient with primary sclerosing cholangitis

We present a case of spindle cell carcinoma of the intrahepatic bile duct that developed in a 34-year-old man with primary sclerosing cholangitis. At the time of presentation, the patient had a 10-year history of primary sclerosing cholangitis. A mass was found in the hepatic hilus and was resected surgically. The tumor was composed predominantly of spindle cells, and a small portion of the tumor appeared to be adenocarcinomatous by histology. Histological and immunohistochemical findings suggested that the spindle cells were epithelial in origin; thus, this tumor was identified as a spindle cell carcinoma. Spindle cell carcinomas arising in the bile duct are extremely rare. Furthermore, the coexistence of spindle cell carcinoma and primary sclerosing cholangitis has not been reported thus far. We were able to follow up the entire course of this rare case. In this case report, we describe the clinicopathological findings of this case and review the literature concerning this type of tumor.