CASE 11683 Published on 08.04.2014

Primary biliary lymphoma


Abdominal imaging

Case Type

Clinical Cases


Pugh L, Twemlow M

North Tyneside General Hospital,
Northumbria Healthcare

82 years, male

Area of Interest Abdomen, Biliary Tract / Gallbladder ; Imaging Technique PET-CT, CT, MR
Clinical History
An 82-year-old gentleman was referred to gastroenterology by his GP with obstructive LFTs of several months duration. He was known to have chronic cholecystitis and gallstones from a previous ultrasound. He had no focal or constitutional symptoms and the physical examination was unremarkable. He was referred for MRCP before elective cholecystectomy.
Imaging Findings
MRCP showed an area of abnormal soft tissue in the porta hepatis causing a stricture of approximately 4cm in the CBD. There were calculi in the gallbladder and lower third of the CBD. Intrahepatic biliary radicals were dilated. This was thought to resemble cholangiocarcinoma.

The patient proceeded to CT which confirmed moderate intrahepatic cholestasis and showed circumferential wall thickening of the proximal CBD with stones in the mid and distal parts. There was associated local lymphadenopathy.
On the basis of these findings the patient underwent ERCP for a presumed diagnosis of cholangiocarcinoma (with ductal calculi).

Biopsies from the ampulla showed diffuse large B-cell lymphoma.

PET CT showed the known common bile duct/ampullary non-Hodgkin's lymphoma and low volume adjacent lymph nodes were markedly hypermetabolic, but showed no evidence of distant disease.

The patient was diagnosed with stage IIA disease, three cycles of attenuated R-CHOP chemotherapy are planned followed by repeat PET to assess the possibility of consolidatory radiotherapy.
Primary biliary lymphoma is extremely rare and the literature almost solely comprises of case reports.
As with the case we present, diffuse large B cell lymphoma (DLBCL) is the most common cause of primary biliary lymphoma and often presents in elderly patients with gallstones present [1].
Gallbladder lymphoma appears to be more common than extrahepatic. Extrahepatic lymphoma is more likely to be associated with a younger patient, it has less regional lymph node involvement and less gallstones. The case we present is the converse to this. Primary biliary lymphoma typically presents with obstructive jaundice [1, 2].

The most common extranodal location for lymphoma is the gastrointestinal system [3].
A study placed the proportion of extranodal lymphoma involving either the liver or biliary system at 0.04% - six cases out of 1476 [4]. Another found two cases of gallbladder lymphoma out of 1168 patients [5].
There are very few reports of primary CBD lymphoma [6].

The radiological appearances of biliary lymphoma are similar to cholangiocarcinoma of the bile duct and most patients receive this as their initial diagnosis [1, 2]. Many patients have gone on to have major biliary surgery for cholangiocarcinoma [2].

We report a case of a patient presenting with obstructive LFTs with CT and MRI examinations showing thickening of the CBD. The imaging and clinical picture was thought to be characteristic of cholangiocarcinoma.
This clinical picture is similar to previous case reports of primary biliary lymphoma [4].

The treatment and prognosis of cholangiocarcinoma and primary biliary lymphoma differ greatly. The two can be extremely difficult to distinguish radiologically [6, 7].
MA Yoon et al have suggested that it may be possible to differentiate biliary lymphoma from cholangiocarcinoma on cholangiography. “…cholangiography shows smooth, mild, luminal narrowing of the extrahepatic bile duct without mucosal irregularity, despite diffuse thickening of the ductal wall on CT/MR images”[2] .

There is no consensus on treatment of primary biliary lymphoma due to the rarity of the disease [2, 7]. The mainstay of DLBCL treatment is chemotherapy [8]. Current chemotherapy regimes may be the primary treatment, with surgery being reserved for complications [9], providing the diagnosis is made preoperatively, which is often not the case.

It has been suggested that, despite the rarity of primary biliary lymphoma, due to the radical difference in treatment from the differentials, namely cholangiocarcinoma, it should be considered as a differential diagnosis [2, 7, 9, 10, 11].
Differential Diagnosis List
Primary biliary diffuse large B cell lymphoma stage II A
Compression from extrabiliary lymphoma
Final Diagnosis
Primary biliary diffuse large B cell lymphoma stage II A
Case information
DOI: 10.1594/EURORAD/CASE.11683
ISSN: 1563-4086