Benign fibrosing disease at the hepatic confluence mimicking Klatskin tumors

Background. Hilar obstructions remain a challenge with regard to diagnosis and treatment. Methods. In the period from 1984 to 1990, 82 patients underwent resective surgery under the presumptive diagnosis of hilar cholangiocarcinoma (Klatskin tumor). The diagnosis was based on the combined appearances on direct cholangiography and ultrasonography in all cases, with the use of various other imaging modalities in some cases. Results. The perioperative findings from an experienced surgical team were usually thought to be compatible with bile duct carcinoma. However, histologic examination of the resected specimens revealed benign fibrosing or localized sclerosing lesions in 11 patients (13.4%). Conclusions. The current state of diagnostic imaging fails as yet to discriminate reliably between benign and malignant hilar lesions. Whereas the immediate therapeutic consequences may be equal (resection followed by hepaticojejunostomy), the late consequences differ in a major way because benign disease has a much better prognosis. In the presence of suspicious hilar obstruction, operable lesions should not be treated by 'palliative' intubational techniques and radiation therapy without a firm diagnosis of malignancy. However, overtreatment (extended liver resection, vascular reconstruction, and liver transplantation) should be avoided as well when a benign lesion has not been ruled out.