Locally advanced mucosa-associated lymphoid tissue (MALT) causing obstructive jaundice
due to involvement of the common bile duct (CBD) is a particularly rare condition.
In the literature there are only 24 case reports of primary CBD lymphoma. To our knowledge
there are no case reports of recurrent locally advanced MALT presenting as biliary
obstruction and diagnosed with single-operator cholangioscopy.
A 67-year-old man with a medical history of Crohn’s disease, duodenal MALT treated
with chemotherapy, and prostate and bladder cancers, was admitted to the hospital
with new-onset jaundice. Liver tests showed a total bilirubin concentration of 25.6 μmol/L,
direct bilirubin 20.5 μmol/L, alkaline phosphatase 414 U/L, aspartate aminotransferase
103 U/L, and alanine aminotransferase 9 U/L. Magnetic resonance imaging of the abdomen
showed moderate to severe intra- and extrahepatic biliary dilation and an obstructing
mass at the level of the mid to distal CBD. The mass appeared to be encasing the CBD.
Endoscopic retrograde cholangiopancreatography (ERCP) was performed. The cholangiogram
revealed a mid-CBD stricture with a round filling defect causing obstruction and proximal
biliary dilatation ([Fig. 1] and [Fig. 2]). For better assessment of the stricture and the filling defect, a SpyGlass probe
(Boston Scientific, Natick, Massachusetts, USA) was introduced ([Fig. 3]), revealing a round, nodular mass in the middle of the bile duct, with associated
luminal reduction, ulceration, and increased vascularity ([Fig. 4], [Video 1]). Biopsies were obtained (SpyBite; Boston Scientific) and a fully covered metal
biliary stent was placed with excellent drainage. Histopathology revealed lymphoid
proliferation infiltrating the mucosa with immunohistochemistry stains compatible
with MALT ([Fig. 5]). The biliary obstruction with secondary jaundice resolved after the placement of
the metal stent and the patient is currently receiving chemoradiation.
Fig. 1 Cholangiogram evidenced a round filling defect in the distal bile duct; scope in
the long position.
Fig. 2 Occlusion cholangiogram showed a mass in the lower part of the bile duct associated
with bile duct stricture and proximal biliary duct dilatation.
Fig. 3 Single-operator cholangioscopy catheter advanced through the stricture into the proximal
bile duct.
Fig. 4 Single-operator cholangioscopy: a large nodule occupying the lumen of the bile duct; b increased vascularity and ulcer associated with the mass in the bile duct.
Fig. 5 Immunohistochemistry stains from single-operator cholangioscopic biopsies consistent
with B-cell lymphoma.
Cholangioscopy revealed a round, friable, nodular mass with increased vascularity.
Because of persistent oozing, continuous irrigation was necessary to enable visualization
of the lesion. The mass with associated exophytic component could be visualized laterally
to the normal-appearing bile duct. Multiple direct cholangioscopy biopsies were taken.
Lymphoma involving the bile duct is rare and is commonly a manifestation of advanced
disease [1]. Biliary obstruction caused by lymphoma occurs in only 1 % – 2 % of all malignant
strictures [2]. Obstructive jaundice, weight loss, abdominal pain, and fever are the most common
symptoms upon presentation [3]. At the time of diagnosis, low-grade MALT lymphomas usually are localized and curable
with local therapy [4]. Lymphoma involving the bile duct is very difficult to diagnose preoperatively [5]. As exemplified by our case, single-operator cholangioscopy can be used to diagnose
biliary lymphoma at the time of therapeutic ERCP.
Endoscopy_UCTN_Code_CCL_1AZ_2AC
