Distal cholangiocarcinomas are rare, but clinicians can use a variety of imaging to diagnose it.
A new case report of a patient with distal cholangiocarcinoma highlights how imaging and histopathology can be used to correctly diagnose the rare and often deadly cancer.
Corresponding author Cathy Kim, MD, of West Virginia University, and colleagues wrote that cholangiocarcinoma only accounts for about 2% of malignancies in the United States, and distal manifestations account for only about 30% of cholangiocarcinomas.
In the case that is the subject of the report, a 68-year-old man came to the emergency department with diffuse abdominal pain, malaise, and nausea. He also reported having had diarrhea for the past 3 weeks. The man had apparent jaundice, which the investigators said had been increasing over the previous 12 hours.
The patient’s past medical history included HIV, hepatitis B, hypertension, and hyperlipidemia.
Laboratory work suggested liver injury, and an ultrasound showed intra- and extrahepatic bile duct dilation. His doctors next opted to use computed tomography (CT), which revealed a periampullary mass of 2.0 cm by 2.7 cm.
The patient was referred to his medical center’s gastroenterology department, where an endoscopic ultrasound and fine-needle biopsy were performed and the malignancy was confirmed.
“Small biliary endoscopic sphincterotomy was performed and a transpapillary biliary stent was placed across the common bile duct stricture,” the authors said.
The patient was then discharged pending follow-up with the hospital’s surgery department.
“Pancreaticoduodenectomy (ie, Whipple resection) was performed revealing metastatic disease with 4 of 22 nodes positive and staging of pT3b N2,” Kim and colleagues said. “Primary tumor resection revealed a moderately to poorly differentiated adenocarcinoma (2.7 cm), centered near the ampulla, with invasion into the duodenum, pancreas, and peri-pancreaticoduodenal soft tissue.”
Subsequent pathology confirmed a pancreatobiliary immunophenotype and a diagnosis of distal cholangiocarcinoma, the investigators said.
“Postoperative course complicated by pancreatic leak, right pleural effusion requiring pigtail placement, and failure to thrive,” they added.
In their discussion of the case, the authors said they were unable to visualize the periampullary tumor upon initial assessment, and while the next step might otherwise have been magnetic resonance imaging, they decided to use CT scans of the abdomen since they did not know the primary source of the issue. They said other types of imaging can also help diagnose and stage bile duct adenocarcinomas, including endoscopic ultrasound, intraductal ultrasound, and cholangioscopy.
The authors said genetic markers can also play an important role in diagnosis. They noted that in a previous study, the marker CK17 was found to be useful to distinguish pancreatobiliary adenocarcinomas from extra-pancreatobiliary non-mucinous adenocarcinomas.
“[T]his is important, as pancreaticobiliary types of ampullary carcinoma have worse survival outcomes compared to intestinal ampullary carcinoma or mixed types,” they wrote. “In our study, CK7 upregulation was used in aiding the diagnosis, as it can be aberrantly upregulated in up to 96% of cholangiocarcinomas.”
The investigators said other factors to look for in cholangiocarcinoma include loss of p53 expression, and they said factors that can increase inflammation can also predispose a patient to malignancy development.
Hathaway QA, Lakhani DA, Balar AB, et al. Distal cholangiocarcinoma: case report and brief review of the literature. Radiol Case Rep. 2022;18(2):423-429. Published online November 22, 2022. doi:10.1016/j.radcr.2022.10.072