Primary Hodgkinʼs Lymphoma Masquerading as a Klatskin Tumor

Abstract

Purpose: Cholangiocarcinoma affects 2–2.8 per 100,000 population per year, and has a 5 year mortality rate of 90%-95%. Current limitations of pre-operative tissue sampling often make a definitive diagnosis elusive necessitagting surgical exploration. We present a case of an obstructing hilar mass, intially thought to be an advanced cholangiocarcinoma, found to be a primary extraductal hilar Hodgkin's lymphoma at laparotomy. Results: A 56 year old male presented with gradual onset of painless jaundice. Initial work- up revealed: AST 464U/L, ALT 548U/L, Alk Phos1697U/L, T.Bili 7.90 mg/dL, and Direct Bili 6.00 mg/dL. Tumor markers including AFP, CEA, LDH, and CA19-9 were unremarkable. Triple phase CT abdomen revealed a common hepatic duct filling defect with intrahepatic ductal dilation without extrahepatic dilation, later confirmed by MRCP. ERCP was then performed revealing a moderate sized stricture involving the common hepatic duct and a short portion of both intrahepatic ducts consistent with a Bismuth II tumor. Brushings were negative for malignancy and temporary biliary stenting was performed for decompression while surgical consultation was obtained for definitive diagnosis. Exploratory laparatomy with choledochoscopy did not reveal cholangiocarcinoma, but rather an obstructing hilar lymph node causing extrinsinc compression of the common hepatic duct. Biopsy revealed lymphocytic rich Hodgkin's lymphoma. Resection and bilateral hepatico-jejunostomy was averted, and the patient was referred to Oncology for treatment. Conclusions: Primary extranodal non-Hodgkin lymphoma of the extrahepatic bile duct mimmicking Klatskin tumor has rarely been reported. Though cholangiocarcinoma accounts for 60–80% of Klatskin tumors; other causes of biliary tree obstruction such as lymphoma should be entertained as treatment may be non-surgical. [figure 1]Figure 1