The Klatskin Tumor That Wasn't: An Unusual Presentation of Sarcoidosis

Abstract

We present the case of a patient who presented with signs and symptoms associated with a Klatskin tumor. After endoscopic retrograde cholangiopancreatography (ERCP) and biopsy, she was found instead to have granulomatous infiltration of the extrahepatic biliary tree consistent with biliary sarcoidosis. The patient was treated successfully with systemic corticosteroids and azathioprine. She later developed cutaneous, lymphatic, and pulmonary granulomatous disease. Isolated biliary disease is a rare initial presentation of systemic sarcoidosis.

Imaging of Biliary Involvement in Sarcoidosis: Computed Tomography, Magnetic Resonance Cholangiopancreatography, and Gadolinium Ethoxybenzyl Diethylenetriamine Pentaacetic Acid-Enhanced Magnetic Resonance Imaging Findings

“…However, the narrowing was in the extrahepatic bile duct, as opposed to our case, in which the stenosis involved intrahepatic bile ducts. Nevertheless, the study by Farooq [31]reporting a jaundiced patient with no pulmonary lesions, and a positive CT finding of an isolated macroscopic hepatic hilar granuloma-is not comparable with the present case, as it concerned a rare presentation of this disease. In contrast, as more frequently reported in hepatobiliary sarcoidosis, our patient was asymptomatic, and showed a widespread involvement of the liver.…”

“…Furthermore, in the present case, an MRCP for evaluating the biliary tree highlighted focal narrowing of intrahepatic bile ducts, without upstream dilations. To the best of our knowledge, there is only one other case in the English literature [ 31 ] reporting a diagnostic MRCP imaging finding characterized by biliary ducts narrowing due to sarcoidosis. However, the narrowing was in the extrahepatic bile duct, as opposed to our case, in which the stenosis involved intrahepatic bile ducts.…”

“…In contrast, as more frequently reported in hepatobiliary sarcoidosis, our patient was asymptomatic, and showed a widespread involvement of the liver. In the literature, other cases of biliary duct stenosis due to hepatobiliary involvement in sarcoidosis are described [ 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 ]. However, all of the above were demonstrated by invasive techniques such as endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous transhepatic cholangiography (PTC).…”

Hepatitis granulomatosa, descifrando un soslayado espectro: enfoue basado en un caso clínico

“…Entre el 50% y 79% de las biopsias hepáticas en pacientes con sarcoidosis muestran evidencia de granulomas hepáticos. Los síntomas más frecuentes son el dolor abdominal y el prurito; solo el 10% cursa con hepatomegalia y menos del 5% se presenta con ictericia aislada (10) ; la hipertensión portal se observa hasta en un 3% de los casos (11,12) . Existen 3 categorías histológicas de sarcoidosis hepática: colestática, necroinflamatoria y vascular (12) .…”

“…Los síntomas más frecuentes son el dolor abdominal y el prurito; solo el 10% cursa con hepatomegalia y menos del 5% se presenta con ictericia aislada (10) ; la hipertensión portal se observa hasta en un 3% de los casos (11,12) . Existen 3 categorías histológicas de sarcoidosis hepática: colestática, necroinflamatoria y vascular (12) . La histología típicamente reporta granulomas epitelioides no necrotizantes, aunque se han descrito granulomas necrotizantes en el contexto de la sarcoidosis (12,13) .…”