Signet-ring cell (SRC) carcinomas are poorly-differentiated malignant tumors that may affect the stomach and the colon, but very rarely occur in other digestive organs1,3. Although SCR distal bile duct cholangiocarcinomas have been very rarely reported2,4,7 we herein first report the occurrence of a SRC hilar cholangiocarcinoma.
A 66-year-old caucasian woman was admitted with a 40-day history of fatigue, anorexia, jaundice, itching and pale stools. She had undergone external percutaneous transhepatic drainage and had been treated for cholangitis with antibiotics. The patient had no palpable masses and her past medical history was unremarkable. Total bilirubin and liver enzymes were mildly elevated, and CBC was normal. Abdominal computed tomography showed a 4.0 x 0.5 cm tumor on the confluence of the right and left hepatic bile ducts with moderately dilated intrahepatic ducts. It also revealed a 2 cm tumor in the left kidney. Additional workup was negative for metastases.
Laparotomy was performed and revealed a hardened mass involving the common bile duct from above the implantation of the cystic duct to the confluence of the right and left hepatic ducts. Resection of the biliary tree was then carried out from the supraduodenal portion of the common bile duct to the first 2 cm of the right and left hepatic ducts, with en-bloc regional lymphadenectomy. A Roux-en-Y jejunal loop was taken to the hepatic hilum and right, left and caudate bile ducts were sewn to a single jejunal loop.
Macroscopic exam of the bile duct tumor revealed a 5.5x0.8cm surgical specimen that was firm and scirrhous tumor located on the confluence of the right and left hepatic ducts (Klatskin tumor). Bile duct confluence had a narrow lumen but no stones or mucin. Gallbladder had no stones or wall thickening.
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