LAUSR

BACKGROUND Biliary papillomatosis (BP) is a rare disease characterized by multiple papillary adenomas in the intrahepatic and extrahepatic biliary tree and has a high risk of malignant transformation. Early diagnosis and treatment for BP are challenges, as it spreads along the biliary tract. A radical resection with an adequate resection margin is advocated in patients with malignant BP. CASE SUMMARY We report a case of BP with malignant transformation in a 52-year-old female patient who presented with emaciation, severe malnutrition, and a T-tube for biliary drainage that was placed for almost 2 years at the time she visited us. The enhanced magnetic resonance imaging of the upper abdomen revealed a neoplasm with a diameter of 15 cm located in the gallbladder area surrounding the common bile duct where a drainage tube was placed. It was the first case with malignant BP involving multiple organs and the abdominal wall and receiving a radical resection. Plastic surgeons helped close the incision by transferring the rectus muscle and external oblique muscle flap. A diagnosis of papillary carcinoma with diffuse malignant papillomatosis of the biliary duct was given. The postoperative course was uneventful and she was asymptomatic and in good physical condition at 35 mo postoperatively with appropriate chemotherapy. CONCLUSION Aggressive surgical treatment with appropriate chemotherapy is advocated for patients with malignant BP even if multiple organ invasion is present.