Gall Bladder and Extrahepatic Bile Duct Lymphomas: Clinicopathological Observations and Biological Implications

Abstract

Lymphomas of the gall bladder and extrahepatic bile ducts are exceedingly rare. We present the clinicopathological features of 19 cases from our files; 14 patients had primary lymphoma (13 involving gall bladder and one involving common hepatic duct), while five had systemic lymphoma on further work-up. Most patients presented with symptoms mimicking cholecystitis. The most common primary lymphoma types were diffuse large B-cell lymphoma (DLBCL), extranodal marginal zone lymphoma (EMZL), B-lymphoblastic lymphoma (B-LBL) and follicular lymphoma (FL). Two cases had features of lymphomatous polyposis, one a case of FL and the second a case of mantle cell lymphoma (MCL), with disease limited to the mantle zones, so-called in situ MCL. Other rare lymphoma subtypes not previously described in this site included the extracavitary variant of primary effusion lymphoma (PEL) and plasmablastic lymphoma (PBL). Patients with DLBCL and EMZL were older (mean age 75.8 years) than those with other subtypes (mean age 47 years) and more likely to have gallstones (60% vs. 12.5%). A comprehensive literature review revealed 36 primary gall bladder and 16 primary extrahepatic bile duct lymphomas. When compared to primary gall bladder lymphomas, those involving the extrahepatic bile ducts present at a younger age (47 years vs. 63 years) usually with obstructive jaundice, and are less often associated with gallstones (17% vs. 50%) or regional lymph node involvement (6% vs. 31%). In conclusion, primary lymphomas of the gall bladder and extrahepatic bile ducts show a broad spectrum of disease types, but in many respects mirror the spectrum of primary lymphomas of the gastrointestinal tract.

Gallbladder lymphoma in a miniature dachshund

“…Histopathological examination with immunohistochemistry in another cat was consistent with a diagnosis of small-cell lymphoma of T cells within the gallbladder, liver and small intestine. In humans, the majority of immunophenotypes were of B-cell lineage and included extranodal marginal zone lymphoma (24%), diffuse large B-cell lymphoma (24%) and follicular lymphoma (15%) [13]. In the dog from our report, the lymphoma was demonstrated to be of B-cell origin, similar to the former case described in a cat and to humans.…”

Primary gallbladder lymphoma presenting as a polyp

“…Our case is the third one reported of a primary follicular lymphoma of the gallbladder but the only report noted presenting as a polyp 8. The other published reports of gallbladder follicular lymphoma were also in women aged between 60 and 80 years.…”

Anaplastic large cell lymphoma (monomorphic post-transplant lymphoproliferative disorder) presenting in gallbladder

“…PTLD may very rarely be isolated to the gallbladder as demonstrated in a case report about a lung transplant recipient who developed EBV-positive polymorphic B-cell PTLD 1 year after transplant [6]. Another more recently described case of PTLD isolated to the gallbladder was reported in a 5-year-old boy, post-cardiac transplant, who developed an EBV-negative PTLD [2]. As with other types of transplantation, B-cell PTLD is much more common than T-cell PTLD (76%-95% vs. 14%, respectively).…”

“…The most common subtypes include diffuse large B-cell lymphoma, marginal zone lymphoma of mucosa-associated lymphoid tissue, follicular lymphoma, and lymphoblastic lymphoma [2]. Rarely, lymphoma may be isolated to the gallbladder with approximately 50 cases reported in the literature [2][3][4][5].…”