Abstract
A 77-year-old man admitted for obstructive jaundice was found in endoscopic retrograde cholangiopancreatography to have stenosis of the inferior bile duct with an irregular wall. The presence of atypical cells in brushing cytology suggested adenocarcinoma. Following endoscopic biliary drainage, we conducted pyloruspreserving pancreaticoduodenectomy, finding a 2.3×1.5cm infiltrating nodular tumor considering histologically of adenocarcinoma on the mucosal side and fascicular atypical spindle cell proliferation (so-called spindle cell carcinoma) and angiosarcoma-like features on the invasive side of the bile duct. The definitive pathological diagnosis was inferior bile duct cancer with sarcomatous change. Despite chemotherapy treatment for multiple liver metastasis detected 6 months postoperatively, he died of the metastatic liver tumor 1.5 years thereafter. Only 15 cases of bile duct cancer with sarcomatous change or spindle cell carcinoma have, to our knowledge been reported in worldwide, and we report this case and review the literature.