Cholangiocarcinoma Complicating Primary Sclerosing Cholangitis: A 24-Year Experience

Abstract

Aim: To report the prevalence and outcome of cholangiocarcinoma arising in primary sclerosing cholangitis for a British tertiary referral centre. Methods: All patients diagnosed with primary sclerosing cholangitis and concurrent cholangiocarcinoma were identified from a prospectively maintained departmental database, and the mode of presentation, management and outcome were determined. Results: Of 370 patients with primary sclerosing cholangitis, 48 patients (13%) were diagnosed with a cholangiocarcinoma within a median time of 0.51 months (range: 0–73.12) from presentation to the unit. Mode of presentation included: inoperable tumours (n = 14); incidental findings in transplant hepatectomy specimens (n = 13); primary sclerosing cholangitis follow-up (n = 9); transplant work-up (n = 5); transplant waiting list (n = 5); suspected tumour confirmed at transplant (n = 1), and incidental finding at cholecystectomy (n = 1). The diagnosis was confirmed by: radiology-guided biopsy (n = 27); MRI (n = 3); CT (n = 2); laparoscopy or laparotomy (n = 2), and frozen section at transplant (n = 1). Management consisted of: transplantation (n = 14, including 1 abandoned); hepatic resection (n = 8), and palliation through stenting (n = 26). The overall median survival of the cohort was 4.9 months (range: 0.09–104.5). Median survival ranged from 2.6 months (range: 0.09–35.3) for palliation to 7.6 months (range: 0.6–99.6) for transplantation and 52.8 months (range: 3.7–104.5) for resection. There was no difference in survival between the transplant and resection groups (p = 0.14). Conclusions: Cholangiocarcinoma is a common finding in primary sclerosing cholangitis and regular screening of this cohort of patients at referring centres is advocated to detect early tumours, as surgical treatment at an early stage offers significantly better outcomes for this cohort of patients.

Risk Factors for Opisthorchis viverrini and Minute Intestinal Fluke Infections in Lao PDR, 2009–2011

“…11 Carcinoembryonic antigen, a marker for colorectal cancer, is elevated in approximately 30% of CCA patients. 12,13 Some potential biomarkers for CCA are trypsinogen-2, 14 mucin-5AC, and the soluble fragment of cytokeratin 19, although none are currently in clinical use. 15,16 The changes in O. viverrini associated with CCA include chronic inflammation and fibrosis combined with nitrative stress from either endogenous and/or exogenous nitrosamines, leading to DNA damage and fixation of mutations.…”

Cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the Study of Cholangiocarcinoma (ENS-CCA)

“…For pCCA, by contrast, jaundice (typically painless) is the most frequent clinical onset 9 . In patients with PSC, CCA can emerge as a rapid deterioration of clinical conditions, dominant stricture during follow-up, during transplantation work-up or waiting list, or as an incidental finding at transplantation 23,[193][194][195][196] . As aforementioned, iCCA occurs more frequently in patients with chronic liver disease (HBV or HCV infection) or parasitic infestation than pCCA 9,197 .…”

The tumorigenic liver fluke Opisthorchis viverrini – multiple pathways to cancer

“…The carbohydrate antigen, CA-19–9, is widely used for diagnosis of CCA, but it lacks specificity as it is also a marker for pancreatic cancer, gastric cancer, primary biliary cirrhosis and in smokers [80]. Similarly, carcinoembryonic antigen (CEA), a marker for colorectal cancer that is sometimes cited as a CCA marker, is only elevated in approximately 30% of CCA patients [83, 84]. In studies using non- O. viverrini induced models of CCA a number of other proteins have been shown to have some potential as biomarkers, including trypsinogen-2 [85], mucin-5AC [86] and soluble fragment of cytokeratin 19 although none of these are currently in clinical use [87, 88].…”