Malignant lymphoma primarily originating from the extrahepatic bile duct is extremely
rare, and preoperative diagnosis is difficult because symptoms and imaging are nonspecific
[1]
[2]. The most common symptoms of primary biliary lymphoma are abdominal pain, weight
loss, fever, and obstructive jaundice [1]
[2]. A total of 23 cases have been reported in literature [1]
[2]
[3]
[4]
[5], and, to the best of our knowledge, this is the fourth case of diagnosis made preoperatively
and the first report of primary biliary lymphoma with acute pancreatitis and cholangitis
as the first clinical manifestations.
A 30-year-old male patient was referred to our hospital for acute pancreatitis. On
admission he had severe abdominal pain, fever, and jaundice. Laboratory findings were:
total leukocyte count, 15 700/µL; C-reactive protein, 7.89 mg/dL; aspartate aminotransferase,
271 U/L; alanine aminotransferase, 428 U/L; alkaline phosphatase, 580 U/L; γ-glutamyl
transpeptidase, 428 U/L; serum total bilirubin, 9.1 mg/dL (direct 8.5 mg/dL); amylase,
951 U/L; and lipase, 1837 U/l. Transabdominal ultrasonography revealed only a dilated
common bile duct (CBD) with sludge in the gallbladder. An urgent endoscopic ultrasound
showed a stricture in the distal portion of the CBD, with proximal bile duct dilatation
([Fig. 1]).
Fig. 1 Endoscopic ultrasound (EUS) image of the stricture in the distal portion of the common
bile duct, with proximal dilatation of the bile duct.
During the same endoscopic session, endoscopic retrograde cholangiopancreatography
(ERCP) and cholangiography confirmed the stricture in the lower portion of the CBD,
with proximal dilatation ([Fig. 2 a]); sphincterotomy and forceps biopsies were carried out ([Fig. 2 b]), and finally a plastic stent was placed for drainage ([Fig. 2 c]).
Fig. 2 Endoscopic retrograde cholangiopancreatography (ERCP) images of the stricture in the
lower portion of the common bile duct: a proximal bile duct dilatation; b forceps biopsies; and c biliary stenting.
Histological examination ([Fig. 3 a]) and immunohistochemistry ([Fig. 3 b – d]) revealed a large B-cell-type malignant lymphoma.
Fig. 3 a Histologic section and b – d immunohistochemistry of the endoscopic biopsy samples.
Total body computed tomography (CT) showed a well-circumscribed, heterogeneous, enhancing
mass in the lower-mid portion of the CBD, without evidence of metastases ([Fig. 4]).
Fig. 4 Abdominal computed tomography (CT) images of the common bile duct mass with no metastasis.
The patient was symptom-free and scheduled for surgical resection. Examination of
the surgical specimen confirmed the diagnosis of large B-cell-type malignant lymphoma,
without metastasis in the lymph nodes included in the resection. The patient was referred
to an oncologist for chemotherapy (rituximab, cyclophosphamide, vincristine, doxorubicin,
and prednisolone [R-CHOP]) and after 6 months of follow-up he remains asymptomatic.
Competing interests: None
Endoscopy_UCTN_Code_CCL_1AZ_2AC
