Case report: xanthogranulomatous cholangitis mimicking cholangiocarcinoma

Presenter: Jenny Q Zhang MD | Rhode Island Hospital Background: Xanthogranulomatous cholangitis is an extremely rare diagnosis and is believed to be an extension of xanthogranulomatous cholecystitis, a benign inflammatory process characterized by lipid-laden macrophages. Here, we report a case of isolated xanthogranulomatous cholangitis mimicking cholangiocarcinoma. Methods: A 72-year-old male who was previously admitted to the hospital for gallstone pancreatitis, cholangitis s/p ERCP with plastic stent placement, and Klebsiella bacteremia underwent interval laparoscopic cholecystectomy with liver biopsy. His pathology revealed chronic cholecystitis and no fibrosis, steatosis or inflammation in the liver biopsy. Postoperatively, he continued to endorse night sweats, unintentional weight loss, and jaundice. He underwent ERCP with exchange of the common bile duct stent multiple times with no alleviation of symptoms. Liver function tests remained mildly elevated (Tbili 1.3, AST 78, ALT 64, Alk Phos 673). CEA was normal (1.1) but CA19-9 was significantly elevated at 160. CT abdomen/pelvis showed new intrahepatic duct dilation and portal vein thrombus. ERCP with SpyGlass revealed right hepatic system underfilling, narrowing of the right intrahepatic duct takeoff, and abnormal biliary tract mucosa characterized by nodularity, scarring, purulence, and decreased vascularity. Cytology of the right main hepatic duct showed atypical cells. Given the constellation of findings, there was high clinical suspicion of early intrahepatic cholangiocarcinoma and he was consented for liver resection. Results: The patient subsequently underwent right hepatectomy and portal lymphadenectomy. Pathology showed xanthogranulomatous cholangitis of large hepatic bile ducts, bile granuloma, hepatic parenchyma with features of large bile duct obstruction, chronic cholestasis, and biliary pattern of fibrosis with bridging septa. Dedicated stains for bacteria and fungi were negative, and there was no evidence of malignancy. He tolerated the procedure well and was discharged home with services on postoperative day 4. Conclusion: We present a patient with initial workup concerning for intrahepatic cholangiocarcinoma. Final pathology showed xanthogranulomatous cholangitis with no malignancy. Interestingly, he did not have xanthogranulomatous cholecystitis. Xanthogranulomatous cholangitis is a very rare phenomenon that can lead to benign strictures of the bile ducts, especially in the setting of recurrent cholangitis.