Korean J Gastroenterol.  2016 Aug;68(2):114-118. 10.4166/kjg.2016.68.2.114.

Primary Biliary Mucosa-associated Lymphoid Tissue Lymphoma Mimicking Hilar Cholangiocarcinoma

Affiliations
  • 1Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. drsong@amc.seoul.kr

Abstract

Primary biliary mucosa-associated lymphoid tissue (MALT) lymphoma is extremely rare. We report a case of primary biliary MALT lymphoma with obstructive jaundice diagnosed by endoscopic biopsy, without surgical intervention. Obstructive jaundice was relieved by endoscopic drainage and endoscopic biopsy was done simultaneously during endoscopic retrograde cholangiopancreatography. Unnecessary surgical intervention can be avoided after pathological confirmation of lymphoma. The patient received radiotherapy, and is alive without any evidence of recurrence or biliary obstruction. Diagnosis of primary biliary lymphoma is very difficult because of its low prevalence. However, it should always be considered as a differential diagnosis, since when an accurate diagnosis is made, unnecessary surgical intervention can be avoided.

Keyword

Lymphoma, mucosa-associated lymphoid tissue; Cholangiocarcinoma; Endoscopic retrograde cholangiopancreatography; Obstructive jaundice; Radiotherapy

MeSH Terms

Biopsy
Cholangiocarcinoma
Cholangiopancreatography, Endoscopic Retrograde
Diagnosis
Diagnosis, Differential
Drainage
Humans
Jaundice, Obstructive
Klatskin Tumor*
Lymphoid Tissue
Lymphoma
Lymphoma, B-Cell, Marginal Zone*
Prevalence
Radiotherapy
Recurrence

Figure

  • Fig. 1. CT scan of abdomen shows enhancing common bile duct (CBD) wall thickening from (A) intrahepatic bile duct (arrow) to (B) CBD (arrows). (C) Magnetic resonance cholangiopancreatography shows completely separated central main bile ducts and dilatations of intrahepatic bile ducts. Common hepatic duct and CBD duct cannot be seen from hilum to intra-pancreatic portion of CBD (arrows). (D) Cholangiography from the ERCP shows severe CBD stricture (arrows) from hilum to proximal CBD with upstream ductal dilatation.

  • Fig. 2. Histopathology and immunohistochemistry showing (A) diffuse infiltration of lymphoid cells (H&E, ×100), (B) B cell proliferation with granulation tissue with lymphoepithelial lesion (H&E, ×200), (C) diffuse immune-positivity for CD20 (×100; monoclonal B cell proliferative lesion) and (D) immune-negativity for CD10 (×100; excluding reactive lymphoid follicle hyperplasia).

  • Fig. 3. Bone marrow aspiration smear (A; Wright-Giemsa stain, ×200) and biopsy (B; H&E, ×100) show a normo-cellular bone marrow without neoplastic lymphoid cell infiltration.

  • Fig. 4. PET shows focal intense F-18 fluorodeoxyglucose uptake (max SUV=6.0) at the hilum of the liver.

  • Fig. 5. After eight months, CT scan shows improvement of lymphoma involvement in common bile duct with decompressed intrahepatic duct dilatation (arrows).


Reference

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