Intrahepatic sarcomatoid cholangiocarcinoma: clinical analysis of seven cases during a 15-year period.

Background: Intrahepatic cholangiocarcinoma with sarcomatous changes is seldom reported and its clinicopathological characteristics are largely unknown.

Methods: Of 466 patients treated in our institution for intrahepatic cholangiocarcinoma from 1991-2005, seven who exhibited sarcomatous features were included in this study.

Results: Most of the tumors were diagnosed in the advanced stage and abdominal pain was the most common presenting symptom. In three cases, the tumors were associated with hepatolithiasis. The tumors frequently appeared as mixed-echoic masses on ultrasonography. They were hypo-attenuated masses on plain computed tomography scans and most appeared with ring-like enhancement after contrast medium injection. Histologically, two of the three resected tumors exhibited complete sarcomatoid change. Four of the liver biopsies also showed only sarcomatous features. Immunohistochemically, tumor cells in the region with sarcomatous features stained positive for both cytokeratin and vimentin antibodies. Follow-up was available in six patients: five died of disease one week to two months after diagnosis; one who remained disease-free 4 years after diagnosis had early stage disease and complete 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 for long term survival in patients with this cholangiocarcinoma variant.