Primary Biliary Mucosa-associated Lymphoid Tissue Lymphoma Mimicking Hilar Cholangiocarcinoma

Seungha Hwang; Tae Jun Song; Seol So; Min Kyung Jeon; Eun Hye Oh; Byoung Soo Kwon; Sujong An; Myung-Hwan Kim

doi:10.4166/kjg.2016.68.2.114

Journal List > Korean J Gastroenterol > v.68(2) > 1007546

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Hwang, Song, So, Jeon, Oh, Kwon, An, and Kim: Primary Biliary Mucosa-associated Lymphoid Tissue Lymphoma Mimicking Hilar Cholangiocarcinoma

Case Report

Korean J Gastroenterol 2016;68(2):114-118.

Published online: 4 October 2016

DOI: https://doi.org/10.4166/kjg.2016.68.2.114

Primary Biliary Mucosa-associated Lymphoid Tissue Lymphoma Mimicking Hilar Cholangiocarcinoma

Seungha Hwang, Tae Jun Song, Seol So, Min Kyung Jeon, Eun Hye Oh, Byoung Soo Kwon, Sujong An, Myung-Hwan Kim

Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

Correspondence to: Tae Jun Song, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea. Tel: +82-2-3010-3914, Fax: +82-2-3010-6517, E-mail: drsong@amc.seoul.kr

Received 18 April 2016 Revised 3 June 2016 Accepted 24 June 2016

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

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

References

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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).

Table 1.

Literature Review of Primary Biliary MALT Lymphoma

Case	Age (yr)/ sex	Preliminary diagnosis	Final diagnosis	ERCP	Pre-operative biopsy/cytology	Treatment	Prognosis	Ref.
1	73/F	NR	Low-grade B cell lymphoma of MALT type	NR	NR	Surgery	Alive after 23 months	3
2	71/F	Bile duct carcinoma	MALT lymphoma of the CBD	＋	Bile cytology:	Surgery	Alive after	4
					negative		40 months
3	71/M	Klatskin tumor	MALT lymphoma of the main hepatic duct junction	NR	NR	Surgery Chemotherapy	Alive after 45 months	5
4	62/M	Cholangiocarcinoma	Marginal zone B cell lymphoma of the MALT type	＋	Brush cytology, biopsy: negative	Surgery	NR	6
5	79/M	Cholangiocarcinoma	Low-grade B-cell lymphoma of MALT	＋	NR	Surgery	NR	7
6	76/F	NR	Non-gastric MALT lymphoma of the biliary tree involving the CBD	＋	Biopsy: negative	Surgery	Died after 33 days	8
Current case	68/M	Klatskin tumor	MALT lymphoma of bile duct	＋	Biopsy: MALT lymphoma	Radiotherpay	Alive after 13 months

MALT, mucosa-associated lymphoid tissue; Ref., reference number; F, female; M, male; NR, not reported; CBD, common bile duct.

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