Primary hematolymphoid malignancies involving the extrahepatic bile duct or gallbladder

Abstract

Primary hematolymphoid malignancies of the extrahepatic biliary tract are rare tumors. We report five cases of primary hematolymphoid malignancies involving the extrahepatic biliary tract. One is a granulocytic sarcoma of the extrahepatic bile duct, another is an extramedullary plasmacytoma of the gallbladder, and the others are two non-Hodgkin lymphomas of the extrahepatic bile duct and one of the gallbladder. The clinical presentations, radiographic studies, and gross findings at surgery have not been a significant help in differential diagnosis. Although a preoperative diagnosis of primary hematolymphoid malignancy is very difficult to reach because of the rarity of this disease, it should be considered, because, if an accurate diagnosis is made before surgical intervention, chemotherapy is the most appropriate treatment. In limited cases mimicking cholangiocarcinoma and gallbladder cancer, surgical resection followed by chemotherapy has a valid role as reasonable treatment for patients.

Primary Malt Lymphoma of the Common Bile Duct

“…Although various lymphoma subtypes have been reported in the literature, the most common subtype is large-cell lymphoma and the most common immunophenotype is B-cell lineage (12, 13). On the other hand, MALT lymphoma of the CBD is extremely rare, and our extensive review of the medical literature disclosed only four previously reported cases (3, 5-7). All four of these cases were elderly patients (age, 62-76 years) with an equal gender distribution.…”

“…As with cholangiocarcinoma, the most common manifestation of the total 23 reported cases of primary CBD lymphoma is acute onset of obstructive jaundice with or without abdominal pain. However, as primary lymphoma accounts for less than 1% of all biliary obstructions and there is a general lack of familiarity with the diagnosis, primary lymphoma is seldom considered in the differential diagnosis (5, 12). Nonetheless, the dramatic difference in the prognosis and treatment of cholangiocarcinoma and primary lymphoma emphasizes the importance of the differential diagnosis in extrahepatic bile duct tumors with obstructive jaundice.…”

“…Since this original description, numerous publications have documented their occurrence in various organs, including the stomach, small bowel, lung, thyroid glands, and salivary glands (3, 4). However, primary occurrence in the common bile duct (CBD) is extremely rare and, to our knowledge, only four cases have been reported in the English-language literature (3, 5-7). We report an extremely rare case of primary low-grade B-cell lymphoma of the MALT type arising from the CBD which clinically and radiologically mimicked cholangiocarcinoma on the preoperative evaluation.…”

A Case of Multiple Myeloma Associated with Extramedullary Plasmacytoma of the Gallbladder Manifesting as Acute Cholecystitis

“…Such involvement may manifest similarly to primary cholangiocarcinoma, primary GB cancer or other common benign inflammatory, infectious, or obstructive diseases of the GB and biliary ducts [ 7 - 14 ]. Therefore, these extramedullary plasmacytomas are not typically considered in the differential diagnosis of biliary diseases, and the majority of cases are detected post-operatively or post-mortem [ 8 ].…”

Primary mucosa-associated lymphoid tissue lymphoma of the hilar bile duct resulting in fluctuant jaundice

“…To our knowledge, to date, only 5 cases reported in the English literature have described such tumors. [ 2 – 6 ] As reported by previous studies, differentiating between a MALT lymphoma of the hilar bile duct and a hilar cholangiocarcinoma (the most common form of bile duct cancer) is challenging.…”