Primary Gallbladder Carcinoma in a Patient with Ulcerative Colitis and Primary Sclerosing Cholangitis (PSC)

Abstract

Case: 46-year-old male with history of ulcerative colitis (UC) diagnosed 28 years ago presented to clinic with three days of abdominal pain, diffuse pruritis and found to have mild jaundice on exam. Initial blood work demonstrated abnormal liver function tests in a mixed cholestatic/hepatotoxic pattern prompting hospital admission. Magnetic resonance cholangiopancreatography showed abrupt narrowing of proximal common bile duct (CBD) with severe dilatation of intra-hepatic bile ducts, suggestive of primary sclerosing cholangitis (PSC). Endoscopic retrograde cholangiopancreatography showed a dominant biliary stricture in the CBD as well as dilatation of proximal CBD and left and right main hepatic ducts. The stricture was treated with dilation and stent placement. CBD brush biopsy results were positive for adenocarcinoma. The patient then underwent a resection of CBD, gallbladder as well as liver biopsy and reconstruction of hepatic ducts. Final pathology revealed poorly differentiated primary gallbladder adenocarcinoma with local tumor extension as well as portal inflammation and fibrosis, consistent with PSC. The patient completed four cycles of chemotherapy and radiation with subsequent re-staging imaging showing no recurrence of disease with normal tumor markers one year after diagnosis. Discussion: PSC is a chronic cholestatic disorder of unclear etiology involving both the intra- and extra-hepatic bile ducts with histology most prominently showing fibrotic and inflammatory changes. Up to 90% of PSC cases are associated with inflammatory bowel disease (IBD), of these 87% have UC. The majority of PSC patients have extensive intestinal and hepatobiliary inflammation that may promote carcinogenesis and are at a high risk for developing hepatobiliary and intestinal neoplasms, mainly cholangiocarcinoma, whereas gallbladder carcinoma (GBC) is rarely reported. The present case demonstrates that prompt recognition of alarming symptoms and aggressive workup led to cancer-free survival despite the diagnosis of GBC, which typically has a very poor prognosis. Therefore, high level of suspicion and attention should be applied to IBD and PSC patients who present with acute signs of possible hepatobiliary pathology as it could represent early signs of hepatobiliary carcinoma.Figure: MRCP showing narrowing of proximal CBD with dilatation of intra-hepatic bile ducts.