Abstract

Introduction. Primary lymphoma of the gallbladder is an extremely rare entity with approximately 50 cases reported so far. In many of these cases the presenting symptoms were mimicking symptomatic gallstone disease and the diagnosis was made postoperatively, especially when the preoperative imaging results were far from suspicious for malignant disease. Patients and Methods. We report a case of primary lymphoma of the gallbladder in an 85-year-old man with gallstone disease, who was admitted for elective cholecystectomy 2 months after an episode of acute cholecystitis and pancreatitis. Histological evaluation of the specimen revealed a small lymphocytic lymphoma of the gallbladder. This type of primary gallbladder lymphoma has not been previously reported. Discussion. The most common primary lymphomas of the gallbladder are MALT lymphomas and diffuse large B-cell lymphomas, although a variety of other histological types have been reported. The association of these lesions with chronic inflammation is the most convincing theory for their pathogenesis. For lesions confined to the gallbladder, cholecystectomy is considered to be sufficient, while supplementary chemotherapy significantly improves prognosis in more advanced disease.

Highlights

  • Primary lymphoma of the gallbladder is an extremely rare entity with approximately 50 cases reported so far

  • Extranodal non-Hodgkin lymphomas represent 40% of all malignant lymphomas, the majority of which involve B-cell clones and originate from the gastrointestinal tract (4%–20% of all non-Hodgkin lymphomas arise in this site) [1]

  • B-monoclonal cells in the peripheral blood were measured lower than 5 × 109/L, being CD20 and CD19 positive and CD5 negative in the immunophenotyping analysis, which excluded the diagnosis of chronic lymphocytic leukemia (CLL) according to the updated WHO definition [4, 5]

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Summary

Introduction

Extranodal non-Hodgkin lymphomas represent 40% of all malignant lymphomas, the majority of which involve B-cell clones and originate from the gastrointestinal tract (4%–20% of all non-Hodgkin lymphomas arise in this site) [1]. Primary lymphoma of the gallbladder without regional spread of the disease and without other organ involvement is extremely rare; to the best of our knowledge about 50 cases have been reported in the English literature so far [2]. The mean age of presentation depends on the specific histological type ranging between 35 and 69 years old [2]. There are only two cases reported in 4- and 5-year-old children, the former being a lymphoblastic lymphoma of the pre-B type and the latter a T-cell non-Hodgkin lymphoma [2, 3]. Primary lymphomas of the extrahepatic biliary tract are even more rare presenting at younger age and most of the times are symptomatic due to bile duct obstruction [2]

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