Intrahepatic Sarcomatoid Cholangiocarcinoma: Clinical Analysis of Seven Cases during a 15-year Period

Background: Intrahepatic cholangiocarcinoma with sarcomatous changes is seldom reportedand its clinicopathological characteristics are largely unknown.Methods: Of 466 patients treated in our institution for intrahepatic cholangiocarcinomafrom 1991-2005, seven who exhibited sarcomatous features were included inthis study.Results: Most of the tumors were diagnosed in the advanced stage and abdominalpain was the most common presenting symptom. In three cases, the tumorswere associated with hepatolithiasis. The tumors frequently appeared asmixed-echoic masses on ultrasonography. They were hypo-attenuated masseson plain computed tomography scans and most appeared with ring-likeenhancement after contrast medium injection. Histologically, two of the threeresected tumors exhibited complete sarcomatoid change. Four of the liverbiopsies also showed only sarcomatous features. Immunohistochemically,tumor cells in the region with sarcomatous features stained positive for bothcytokeratin and vimentin antibodies. Follow-up was available in six patients:five died of disease one week to two months after diagnosis; one whoremained disease-free 4 years after diagnosis had early stage disease andcomplete surgical resection.Conclusions: Intrahepatic sarcomatoid cholangiocarcinoma is a rare but aggressive malignancy.The diagnosis needs to be confirmed by immunohistochemical study.Early detection and complete surgical resection offer the only chance forlong term survival in patients with this cholangiocarcinoma variant.