Xanthogranulomatous Cholecystitis and Cholangitis: A Single Case Study

Abstract

Purpose: Xanthogranulomatous cholecystitis (XGC) is a benign inflammatory disease of the gallbladder. The incidence of XGC is 1% to 2% and clinically mimics chronic cholecystitis. Although the incidence suggests that XGC is an unusual rather than rare condition, cases from the United States have seldom been reported. Xanthogranulomatous cholangitis is an even rarer entity, with a prevalence of 0.6%. It manifests as biliary strictures with associated wall thickening, and is indistinguishable from infiltrative cholangiocarcinoma. We present a unique case of xanthogranulomatous cholangitis in the setting of XGC. Case: A 37 yo gentleman presented with weight loss and diarrhea. Exam revealed jaundice and right upper quadrant (RUQ) tenderness. The bilirubin was elevated (4.4), prompting an MRCP that revealed an obstructive stone in the common bile duct (CBD). Because of adhesions and gallbladder necrosis on laparoscopy, the patient underwent an open cholecystectomy. Stones were removed from the CBD and the common hepatic duct (CHD) was found to be full of debris. A t-tube was placed and a JP drain was left in the gallbladder fossa. Pathology from the gallbladder revealed XGC. Worsening jaundice (bilirubin 13.8), RUQ pain, and vomiting complicated the post-operative course. T-tube cholangiogram revealed intrahepatic biliary ductal dilation and stricture of the CHD, suggestive of xanthogranulomatous cholangitis. A bile leak from the CBD was also identified and a percutaneous biliary drain was placed. The patient was also treated for acute pancreatitis secondary to an obstructing stone. The remainder of the hospital course was unremarkable and he was discharged with instructions for a follow-up ERCP. The patient was subsequently lost to follow-up. Discussion: In 1981, Goodman and Ishak described the first cases of XGC. While this entity has been frequently documented in India and Japan, where the incidence of gallstones is higher, only one case report has been published in the United States in the last 15 years. This entity is overlooked because it clinically resembles chronic cholecystitis. However, it can be easily confused with gallbladder cancer, a much graver condition. Similarly, information about xanthogranulomatous cholangitis has been limited to single case reports describing strictures mimicking malignancy. Although the majority of patients with painless jaundice are ultimately diagnosed with cancer, 5-10% harbor benign disease. It is impossible to grossly differentiate these cases; histopathology is necessary. Because XGC and xanthogranulomatous cholangitis can both be confused for malignant conditions, we must be aggressive in identifying and reporting these cases and variations thereof.