Baylor University Medical Center Proceedings April 2017 - 191

a

b

Figure 2. (a) A hematoxylin and eosin stain demonstrates distorted cells, with arrows demarking an entrapped gland. (b) The biopsy demonstrates positive CD20
staining. Overall findings were consistent with B-cell lymphoma.

area of the hilar bile duct without metastatic disease. Pretreatment evaluation is under way, with plans to initiate rituximab,
cyclophosphamide, doxorubicin, vincristine, and prednisone
(R-CHOP) chemotherapy.
DISCUSSION
Non-Hodgkin's lymphoma resulting in obstructive jaundice
is primarily caused by extrahepatic lymphoma compressing the
bile duct, causing a mass effect. Very rarely is obstructive jaundice due to primary bile duct lymphoma, as in this vignette
(4). On imaging, intra- and extrahepatic bile duct dilation was
noted. Essentially identical radiologic findings may be found
in the setting of cholangiocarcinoma.
Adequate biopsy is needed for definitive diagnosis, as the
management and prognosis for cholangiocarcinoma and lymphoma are notably different (5). Treatment for cholangiocarcinoma is surgical resection or gemcitabine-based chemotherapy.
Lymphoma is more chemoresponsive, and management utilizes
the R-CHOP regimen. In addition, a diagnosis of lymphoma
carries a better overall prognosis than cholangiocarcinoma.
Biliary obstruction in the setting of lymphoma correlates with

April 2017

advanced disease, only emphasizing the importance of rapid
diagnosis and initiation of correct treatment. As lymphoma is an
AIDS-defining illness, its diagnosis in an HIV-infected patient
is not only important for initiation of appropriate treatment
but also serves a prognostic purpose. The diagnosis indicates an
immunocompromised state, regardless of CD4 count, and the
need for careful oversight of the patient's overall management.
1.

2.

3.

4.

5.

Chi M, Tandra P, Jallad B, Iroegbu N. A primary biliary diffuse large
B-cell lymphoma mimicking Klatskin tumor in an HIV patient. Onkologie
2011;34(10):555-556.
Eliason SC, Grosso LE. Primary biliary malignant lymphoma clinically
mimicking cholangiocarcinoma: a case report and review of the literature.
Ann Diagn Pathol 2001;5(1):25-33.
Mikail C, Sefa T, Tamer K, Anil SO, Gulcin Y. Primary extrahepatic
bile duct lymphoma mimicking Klatskin's tumor, dramatic response to
chemotherapy. Int J Surg Case Rep 2015;8C:147-149.
Sharma J, Garg PK, Jain D, Bakshi S, Pandey D. Primary Hodgkin's
disease of the common bile duct: a case report and review of literature.
J Gastrointest Cancer 2014;45(Suppl 1):218-221.
Ghose A, Sethi N, Li G, Chaudhary R. Chemotherapy versus surgery in
primary B-cell lymphoma masquerading as Klatskin tumor-a diagnostic
and therapeutic dilemma. Am J Ther 2011;18(6):e255-e257.

Bile duct lymphoma disguised as cholangiocarcinoma

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