Abstract:A rare case of a huge carcinosarcoma of the gallbladder is presented. Despite a tumor thrombus in the portal vein, this tumor was resected successfully by extended right hepatic lobectomy with right caudate lobectomy and gastroduodenectomy. However, hepatic metastases developed rapidly, and the patient died only 3 months after surgery. Macroscopically, a whitish tumor filled the body and neck of the gallbladder and involved the right lobe and left medial segment of the liver, stomach, and duodenum. Microscopically, the neoplasm consisted of both carcinomatous and sarcomatous components. The former contained adenocarcinoma and squamous cell carcinoma, which were observed in the wall of the gallbladder. The latter comprised the bulk of the mass and contained malignant cartilage and osteoid. ImmunohistochemicaIly, the sarcomatous cells reacted to antibodies for epithelial membrane antigen and cytokeratin, but were negative for vimentin antibody. Although stromal differentiation into osteoid and cartilage was noted, the sarcomatous component was felt to be derived from mesenchymal metaplasia of the carcinomatous cells. Only 14 cases of carcinosarcoma of the gallbladder have been reported in the English language literature since 1967.