抄録
A 55-year-old woman was diagnosed as having Gardner syndrome’s at the age of 28. Blood chemistry analysis showed repeated episodes of liver dysfunction and inflammatory worsening from early 2013. MRCP revealed intrahepatic bile duct dilatation. Abdominal CT revealed a low-density lesion in the porta hepatis causing biliary obstruction. Further investigation by ERCP revealed a post-papilloplasty state and a sessile lesion in the lower bile duct. We obtained biopsy specimens from the two different lesions and placed a drainage tube at the portal hepatis. Transnasal endoscopy with a slim endoscope allowed us to visualize the irregular lesion in the bile duct at the hilum. There was obstruction of the bile duct at the porta hepatis and a type IIa lesion in the lower bile duct. Endoscopic biopsy of these separate lesions showed identical features, those of an adenocarcinoma. Surgery was performed. Histopathological examination of the resected specimens revealed papillary adenocarcinoma of the bile duct, pat BlcsiA, m/s (-) , pHinf0, pGinf0, pPanc0, pDU0, pPV0, pA0, pN0, pHM0, pDM0, pEM0, ly0, v0, ne0, fStage I. The two lesions were histopathologically connected.
We report a rare case of superficial widely spreading carcinoma of the bile duct occurring 27 years after the diagnosis of Gardner’s syndrome.
