Primary Hepatic Non-Hodgkin's Lymphoma: An Enigma Beyond the Liver, a Case Report

We have discussed a unique presentation of primary diffuse large cell B-cell non-Hodgkin (DLBC NHL) hepatic lymphoma involving the porta hepatis and biliary confluence causing obstructive jaundice with contiguous soft tissue involvement of the right lobe of liver extending up to the right renal cortex. This appears to be the only case in literature where primary hepatic lymphoma has shown contiguous localized intra- and extrahepatic tumor infiltration. A 67-year-old gentleman presented with history of significant loss of appetite and weight in 2 months with associated progressive painless cholestatic jaundice. Physical evaluation revealed normal vitals with pallor, deep icterus, scratch marks over the abdomen, generalized muscle wasting, grade II clubbing and a palpable non-tender liver with a globular, firm mass beneath the liver. He had a total serum bilirubin of 15.9 mg/dL and direct bilirubin of 9.24 mg/dL. His liver enzymes were moderately elevated with raised serum creatinine and dyselectrolytemia. Serology for enterohepatic viruses was negative. Contrast-enhanced magnetic resonance imaging (CEMRI) showed poorly enhancing multiple soft tissue masses in both lobes of liver with the largest mass involving, biliary confluence and porta hepatis causing right bile duct and portal vein encasement. The mass occupied the posterior right lobe and extended to the inferior surface of liver with contiguous invasion of the right renal upper pole cortex. The mass was associated with a retracted liver capsule in the involved segments and delayed enhancement, mimicking a cholangiocarcinoma. Tissue biopsy revealed hepatic DLBC type NHL and patient was subsequently treated with a CHOP-R (cyclophosphamide-doxorubicin-vincristine-prednisolone/rituximab) regimen, on which he has shown non-progressive disease at 1-year follow-up. DLBC NHL of the liver is a very rare tumor with propensity for isolated involvement of the liver and minimal extrahepatic spread. This case shows many interesting features such as obstructive jaundice for 2 months, porta hepatis involvement and tumor infiltration up to the right renal parenchyma. We have illustrated various imaging findings which should be considered when evaluating such a lesion to help differentiate it from cholangiocarcinoma. The literature is extensively reviewed. The case demonstrates relevant diagnostic parameters for physicians, radiologists and oncologists who are likely to encounter patients with tumor-induced obstructive jaundice in their daily practice.