Primary Biliary Malignant Lymphoma Clinically
Mimicking Cholangiocarcinoma: A Case Report and
Review of the Literature
Stephen C. Eliason, MD and Leonard E. Grosso, MD, PhD
Primary lymphomas of the liver and biliary tract are rare tumors. We describe an
unusual case of a diffuse large B-cell lymphoma arising in the extrahepatic bile ducts
with local extension to involve the intrahepatic bile ducts. The patient presented
solely with obstructive biliary symptoms. The clinical presentation, radiographic
studies, and gross findings at surgery suggested that this patient had a Klatskin tumor
(cholangiocarcinoma arising at the junction of the left and right hepatic ducts).
While rare, the difference in initial patient management emphasizes the importance
of including malignant lymphoma in the differential diagnosis of obstructive biliary
lesions.
Ann Diagn Pathol 5: 25-33, 2001. Copyright © 2001 by W.B. Saunders Company
Index Words: Lymphoma, hepatobiliary, Klatskin tumor
H
EPATOBILIARY involvement by non-Hodg-
kins lymphoma is almost always secondary to
a known, existing systemic lymphoma or leukemia.
Secondary involvement of the liver has been com-
monly reported for chronic lymphocytic leukemia
or small lymphocytic lymphoma (3.6% of hepatic
lymphomas), follicular lymphomas (22.4%), dif-
fuse large cell lymphomas (18.2%), and Hodgkin’s
disease (35.7%).
1
While less frequent, hepatic in-
volvement by many other categories of systemic
lymphoma, including lymphoblastic lymphomas
and peripheral T-cell lymphomas, has been
reported.
1
In contrast to secondary involvement, primary
malignant lymphomas, either Hodgkin’s or non-
Hodgkin’s lymphoma, arising in the liver or biliary
system are rare. Patients with primary hepatic lym-
phoma frequently have “B” symptoms and gener-
ally present with abdominal pain and/or an ab-
dominal mass.
2,3
In these patients hepatomegaly is
the most common physical finding. Generally, bil-
iary tumors are accompanied by jaundice with evi-
dence of biliary obstruction.
4
We present an un-
usual case of non-Hodgkin’s lymphoma of the
extrahepatic bile ducts presenting solely with ob-
structive jaundice. Clinical presentation and labo-
ratory and radiographic findings mimicked a
Klatskin tumor.
Case Report
A healthy, 41-year-old white woman presented
with a 2 month history of pruritus that was initially
attributed to a food allergy. There was no history of
hepatobiliary disease, ulcerative colitis, alcohol or
tobacco abuse, or recent weight loss. The patient’s
past medical and surgical history were unremark-
able. Therapy with benadryl and topical corticoste-
roids was started. After 2 months, no improvement
was noted and the patient had become jaundiced.
Serologic studies for hepatitis viruses A, B, and C as
well as Epstein-Barr virus were negative. The pa-
tient’s physical examination was unremarkable.
There was no evidence of hepatosplenomegaly or
enlarged lymph nodes. Pertinent laboratory data
from the time of admission are summarized in
Table 1.
Abdominal ultrasound showed no abnormalities
of the liver or gallbladder. Abdominal computed
tomography showed biliary obstruction without
identifiable masses. Endoscopic retrograde cholan-
giopancreatography failed to show the hepatic
From the Department of Pathology, Wilford Hall Medical Center, TX;
and the Department of Pathology, Saint Louis University School of
Medicine, Saint Louis, MO.
Address reprint requests to Leonard E. Grosso, MD, PhD, Department
of Pathology, Saint Louis University School of Medicine, 1402 S Grand
Blvd, Saint Louis, MO 63104.
Copyright
©
2001 by W.B. Saunders Company
1092-9134/01/0501-0008$35.00/0
doi:10.1053/adpa.2001.21483
25
Annals of Diagnostic Pathology,
Vol 5, No 1 (February), 2001: pp 25-33