An Unexpected Diagnosis from ERCP for Ascending Cholangitis and Ampullary Mass: Extranodal Diffuse Large B-Cell Lymphoma

Abstract

Diffuse large B cell lymphoma (DLBCL) is a histological subtype of non-Hodgkin lymphoma (NHL) that accounts for approximately 25% of all NHL cases. DLBCL usually presents as an enlarging mass, most commonly as nodal enlargement in the neck or axilla. Systemic B-symptoms are often reported, including severe night sweats, fever, and weight loss. Extranodal disease has been reported in up to 30 percent of cases, and the diagnosis of DLBCL is most commonly made with excisional lymph node biopsy. A 73 y/o F with a PMH of anemia presented with painless jaundice, abdominal pain, and weight loss. Physical was remarkable for jaundice and sclera icterus. Labs were positive for a WBC of 18.4, ALT of 73, AST of 57, ALKP of 786, total bilirubin of 3.7, direct bilirubin of 3.2, LDH of 247, and lipase of 859. US abdomen showed marked intra- and extra-hepatic biliary dilation, gallbladder distention, and abrupt termination of the CBD in the pancreatic head. CT scan confirmed biliary dilation with extensive peritoneal metastases or lymph nodes. A CA 19-9 level was 8894. An ERCP with cytology brushing and ampulla biopsy revealed an ampullary mass, a 1.5 cm dilated intrahepatic biliary tract, and a 5 cm distal and mid-common bile duct stricture beginning at the ampulla. A PET-CT scan was obtained, which showed abnormal uptake in posterior mediastinal, right axillary, right cardiophrenic, mesentery, and retroperitoneum lymph nodes, as well as in the right liver lobe and the pancreatic head and tail. Cytology brushing revealed atypical cells, and ampullary biopsy revealed DLBCL. This case of DLBCL was unique for multiple reasons. The initial symptoms of painless jaundice, abdominal pain, subjective fevers, and recent weight loss are not typical presenting symptoms of DLBCL; no rapidly enlargement mass or nodal enlargement of the neck and axilla was present. Her symptoms could have been explained solely by her ascending cholangitis. It was also remarkable how the clinical presentation resembled pancreatic adenocarcinoma but was actually due to a DLBCL with significant distant metastasis. This less common extranodal presentation would not have been expected given her complaints, as additional symptoms or a diagnosis would have presumably been previously suspected. Lastly, the majority of DLBCL diagnoses are made with excisional lymph node biopsy, but this case's diagnosis was interestingly made from an ampullary mass biopsy from an ERCP for ascending cholangitis.