Localized primary sclerosing cholangitis mimicking as a Klatskin tumour: report of three cases / 中华肝胆外科杂志

ABSTRACT

Objective To report three cases of localized primary sclerosing cholangitis (PSC) mimicking a hilar cholangiocarcinoma (Klatskin tumor) and to summarize their clinical characteristics and the ways to differentiate them through a literature review.Method The clinical data of three patients with localized PSC mimicking a hilar cholangiocarcinoma were retrospectively analyzed.The characteristics of laboratory tests and imaging examination were reviewed,and therapy and prognosis were discussed.Results The three patients were all diagnosed to have a hilar cholangiocarcinoma preoperatively,but the diagnosis of PSC was confirmed by histopathology post-operatively.All the three patients had elevated CA19-9,2 patients had elevated anti-nuclear antibody (ANA) and 2 patients had elevated IgG.All the three patients underwent surgical resection and histopathological study showed chronic inflammation of the hilar bile ducts and cholangitis of the intrahepatic portal area.The three patients were followed up from 7 months to 8 years with no symptoms.Conclusions Localized PSC is rare and it can casily be misdiagnosed as a hilar cholangiocarcinoma.Biopsy before surgery is helpful for the differential diagnosis but it is difficult to get a good biopsy sample.Surgical resection is an effective treatment.