Hepatic Sarcoidosis Mimicking Klatskin Cholangiocarcinoma

Abstract

A 57-year-old African-American man presented with progressive obstructive jaundice, pruritus, and weight loss. Investigations were notable for elevated liver canalicular enzymes with negative serologies for viral hepatitis, autoimmune, and metabolic liver diseases. Serum CA 19–9, CEA, and AFP were within normal limits. ERCP demonstrated a complex stricture of common hepatic duct (CHD) at the confluence with proximal extension into both intrahepatic ducts (Bismuth-Corlette classification IV) (Figure 1). Intraductal brushings across the stricture were negative for malignancy. MRI revealed a lesion measuring 2.5 × 2.5 × 2.8 cm encasing the CHD (Figure 1). At EUS, a 2.4-cm lesion was identified surrounding the CHD (Figure 3). Cytology from FNA of the lesion was negative for malignancy. Liver biopsy revealed mild lobular hepatitis without granuloma or fibrosis. CT scan of the chest showed a normal lung parenchyma without mediastinal lymphadenopathy. Given a lesion that was highly suspicious for Klatskin cholangiocarcinoma without evidence of metastasis, he underwent an exploratory laparotomy with right hepatic lobe resection and Roux-en-Y left hepaticojejunostomy. Final pathology revealed non-caseating granulomatous inflammation forming a 3.5 cm mass in the hilar soft tissue adjacent to CHD without any evidence of malignancy or infection, consistent with sarcoidosis. In this report, we describe a unique case of hepatic sarcoidosis presenting as obstructive jaundice due to the isolated involvement of CHD and bilateral intrahepatic ducts, mimicking Klatskin cholangiocarcinoma. No involvement of other organs was identified. To our knowledge, this is the first such case report in the literature. It highlights the great difficulty in establishing a definitive diagnosis for the etiology of a biliary stricture without performing an exploratory laparotomy despite the application of multiple pre-operative radiographic modalities and tissue sampling techniques. If hepatic sarcoidosis had been diagnosed pre-operatively, a therapeutic trial of corticosteroids might have been attempted initially.[figure 1]Figure