Primary Non-Hodgkin's Lymphoma of Different Sites of the Biliary Tree Masquerading as Cholangiocarcinoma Diagnosed Accurately with EUS-FNA Sparing Unnecessary Surgery - Four Cases with Review of Literature
Purpose: Primary non-Hodgkin's lymphoma (PNHL) of biliary tree is an extremely rare condition and it presents as cholangiocarcionma (CC). Differentiating between the two entities is particularly important from the point of prognosis and management.
Methods: The demographic characteristics, clinical, laboratory, radiological and cytological findings of 4 patients diagnosed with PNHL of biliary tree during 1 year are presented and analyzed together with the cases gathered from a review of the English literature.
Results: All the cases in literature along with our 4 cases with PNHL mimicked CC clinically. Almost all patients had vague primary presenting symptoms of abdominal pain, weight loss and jaundice. Beginning of symptoms was with in the previous 4-8 weeks. The clinical findings that favored PNHL from CC were manifestation of B-symptoms, larger size of the lesion, less occurrence of invasion to the vessels and no distant metastasis at the time of diagnosis. All of our 4 cases had high to extremely high bilirubin and normal or slightly elevated CA 19-9. The masses in our patients had variable location, one was involving biliary hilum (mimicking Klatskin's tumor), two were located the mid common bile duct (CBD) and the last one was involving the distal CBD at the head of the pancreas. Brushing of biliary stricture for cytology was negative in all 4 cases. The diagnosis of PNHL in all of our cases was made by EUS Fine needle aspiration (FNA) with out any complication. Flowcytometry confirmed the diagnosis of PNHL. All 4 patients were managed with chemotherapy / radiation and spared them from having major surgery.
Conclusion: Despite the difficulty in differentiating PNHL of the biliary tree from CC, the diagnosis of PNHL can be suspected based on clinical, laboratory and radiological features and can be confirmed accurately by EUS FNA. The accurate diagnosis will have a major impact on the prognosis and management; furthermore, it can spare the patient from unnecessary surgery.© The American College of Gastroenterology 2012. All Rights Reserved.