To Depict a Case of Concomitant Diagnosis of PSC and Cholangiocarcinoma (CCA) in a Patient with History of Surgically Treated UC for 25 Years

Abstract

Purpose: To depict a case of concomitant diagnosis of PSC and cholangiocarcinoma (CCA) in a patient with history of surgically treated UC for 25 years. Methods: A 72-year-old Indian male with history of UC for 25 years and colon cancer s/p proctocolectomy presented with epigastric pain. He was complaining of fevers and chills for 10 days and developed epigastric pain for last 3 days. Pain was 8/10 in intensity, non-radiating, sharp, and was associated with decreased appetite. He denied any nausea, vomiting, diarrhea or constipation or weight loss. His LRTs showed cholestatic picture, of note for past 5 years they were within normal range. A CT scan of abdomen showed intrahepatic biliary ductal dilatation the transition to normal-caliber at the porta hepatis and a questionable filling defect at the point of transition within the CBD. A stone or mass could not be excluded. Results: MRCP showed marked bilobar intrahepatic biliary ductal dilatation with lack of identification of the common hepatic, ERCP showed small filling defects in the CBD and stricture was noted in the distal right hepatic duct proximal to the hilum. Left hepatic duct appeared irregular and dilated likely secondary to PSC. CA-199 was abnormal with a level of 8589 and brush cytology was negative for malignancy. Patient's family opted to continue treatment in India. There, he underwent percutaneous biopsy and PET scan showed CCA with metastasis. Conclusion: PSC is a cholestatic liver disease characterized by multiple fibrotic strictures of the intra- and extrahepatic biliary tree. CCA is the most feared complication of PSC and the occurrence of CCA leads to a poor prognosis for PSC patients. The prevalence of CCA in patients with PSC is reported to be approximately 7-15% in the USA. The average follow-up period between PSC and CCA diagnosis is relatively short (2.6 to 3.5 years). Also, PSC patients with IBD are less likely to develop CCA. In the U.S. and Europe, PSC is the major risk factor associated with the development of CCA, usually leading to death within a few months of diagnosis. The highest risk is in newly diagnosed patients within the first year, and thereafter, the incidence of CCA is about 1% per year. Unlike CCA without PSC, it is difficult to diagnose CCA in patients with PSC because PSC itself shows similar diffuse radiographic findings of bile duct derangement, and brush cytology and biopsy often fail to detect cancer cells because of the desmoplastic nature of CCA.