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Sarcomatoid carcinoma of the common bile duct (CBD) is an extremely rare malignant neoplasm, which is characterized by the presence of carcinomatous and sarcomatous components. We report a case of sarcomatoid carcinoma arising in the distal CBD. The patient was a 68- year-old woman who presented with abdominal pain. The computed tomography and endoscopic ultrasonography revealed a polypoid mass in the distal CBD. The resected specimen showed a polypoid mass with a narrow stalk in the distal CBD which was confined to the mucosa. The cut surface revealed a gray-whitish solid mass with focal hemorrhage and necrosis. Microscopically, the tumor was composed of carcinomatous and sarcomatous components without any heterologous elements. The sarcomatous area predominently consisted of pleomorphic spindle cells. The carcinomatous component was an adenocarcinoma. On immunohistochemistry, cytokeratin was coexpressed in the carcinomatous and sarcomatous components but vimentin was expressed exclusively in the sarcomatous component. The patient has been doing well for one year postoperatively.

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