Biliary Schwannoma That Required Differentiation from Bile Duct Cancer

Schwannomas are benign tumors originating from Schwann cells, which are the main component of the neural sheath. Biliary schwannomas are extremely rare. We report the case of a 78-year-old man who presented with no abdominal symptoms or jaundice. CT imaging showed a hyperdense mass extending along the extrahepatic bile duct, and the upstream bile ducts were dilated. We performed extrahepatic bile duct resection under a preoperative diagnosis of the extrahepatic bile duct cancer. A histopathological examination of the resected specimen revealed that the tumor consisted of spindle cells which exhibited a palisading arrangement. Immunohistochemical staining was positive for protein S-100 and vimentin. Based on these pathological findings, we diagnosed the patient with schwannoma of the extrahepatic bile duct. Our search of the relevant literature revealed 19 case studies of biliary schwannomas. In our case, the surgical findings showed that the tumor was noninvasive and mobile. During surgery, a fast frozen section analysis was performed, and no malignant findings were observed. These results enabled us to avoid extrahepatic bile duct resection with major hepatectomy. We experienced a case of biliary schwannoma that was difficult to distinguish from bile duct cancer.