Outcome after resection for perihilar cholangiocarcinoma in patients with primary sclerosing cholangitis: an international multicentre study

Abstract

Background: Resection for perihilar cholangiocarcinoma (pCCA) in primary sclerosing cholangitis (PSC) has been reported to lead to worse outcomes than resection for non-PSC pCCA. The aim of this study was to compare prognostic factors and outcomes after resection in patients with PSC-associated pCCA and non-PSC pCCA.Methods: The international retrospective cohort comprised patients resected for pCCA from 21 centres . Patients operated with hepatobiliary resection, with pCCA verified by histology and with data on PSC status, were included. The primary outcome was overall survival. Secondary outcomes were disease-free survival and postoperative complications.Results: Of 1128 pCCA patients, 34 (3.0%) had underlying PSC. Median overall survival after resection was 33 months for PSC patients and 29 months for non-PSC patients (p = .630). Complications (Clavien-Dindo grade 3) were more frequent in PSC pCCA (71% versus 44%, p = .003). The rate of posthepatectomy liver failure (21% versus 17%, p = .530) and 90-day mortality (12% versus 13%, p = 1.000) was similar for PSC and non-PSC patients. Conclusion:Median overall survival after resection for pCCA was similar in patients with underlying PSC and non-PSC patients. Complications were more frequent after resection for PSC-associated pCCA, with no difference in postoperative mortality.

EASL Clinical Practice Guidelines on sclerosing cholangitis

“…The complication rate was higher in PSC but it did not affect post-operative mortality. 328 Surgical resection should be considered in cases where liver transplantation is not possible. Careful evaluation, risk assessment and selection of patients before surgery is crucial and should be carried out by the multidisciplinary team.…”

Cholangiocarcinoma cell proliferation is enhanced in primary sclerosing cholangitis: A role for IL‐17A

“…Despite the fact that our study identified an increased proliferation in PSC-CCA compared with sporadic CCA, a multi-center study recently found no difference in survival after resection between perihilar PSC-CCA and sporadic CCA. 20 Various factors could be involved, including the younger age of PSC-CCA patients, which means they are probably physically more fit with less comorbidity than older patients. The median age of diagnosis of CCA in PSC patients is about 20 years earlier than patients with CCA without PSC.…”

Treatment of cholangiocarcinoma in patients with primary sclerosing cholangitis: a comprehensive review

“…72 In an era of transplantation oncology, in which PSCassociated pCCA has been considered unresectable, recent data on patients with PSC undergoing liver resection are understandably limited. 66 71 A North American multicentre study from 2018 reported a similar outcome in patients with resectable pCCA with and without PSC, with a 5-year overall survival (OS) and disease-free survival (DFS) of approximately 20%. 73 Lymph node involvement, vascular invasion, low tumour differentiation and higher age are associated with worse OS.…”

“…65 66 Even so, radicality is difficult to obtain, morbidity is high and long-term survival remains low with a reported 5-year survival ranging from 20% to 40%. [65][66][67][68] Patients with resected BTC are offered 6 months of adjuvant chemotherapy with capecitabine, according to current guidelines. 69 70 Due to the underlying parenchymal liver disease and the propensity of skip cancer lesions, liver resection in PSC-pCCA is even more challenging, often resulting in narrow margins despite extended resection and the high morbidity and mortality that it entails.…”