Journal Club: Unusual case of biliary neuroendocrine tumor

This research article is published in the Case Reports in Clinical Radiology, published by Scientific Scholar.

Neuroendocrine tumours (NETs) affecting the extrahepatic bile ducts are extremely uncommon, with limited documented cases in the literature. Biliary NETs often present with obstructive jaundice. They are commonly misdiagnosed as cholangiocarcinoma due to their similar clinical presentation. This case report showcases the imaging findings of a neuroendocrine tumour in the common hepatic duct in a patient presenting with obstructive jaundice.

Discussing the case report-

A 33-year-old male with no known comorbidities presented with complaints of upper abdominal pain and discomfort, dyspepsia, dark-colored urine, yellowish discolouration of eyes, pruritus, and clay-colored stool of 1-month duration. There was no history of fever, abdominal lump, or change in bowel habits. The patient’s laboratory results showed an elevated bilirubin level and features of obstructive jaundice, with an increase in liver enzyme levels. However, the other laboratory parameters, such as complete blood count, prothrombin time, amylase, albumin, and viral markers, were found to be within normal limits.

Magnetic resonance cholangiopancreatograph,y followed by contrast administration, showed a well-defined lesion in the region of the proximal common hepatic duct which was mildly hyperintense. The lesion was seen extending into the adjacent portions of the hepatic ducts, resulting in dilation of the proximal intrahepatic biliary tree.

A diagnosis of cholangiocarcinoma was raised. The patient was scheduled to undergo an extended right hepatectomy. After ensuring adequate left liver lobe volume, the patient proceeded with an extended right hepatectomy and hepaticojejunostomy.

Pathological and immunohistological examination led to a final diagnosis of grade 2 periductal infiltrating neuroendocrine with no lymphovascular infiltration. A whole-body positron emission tomography-computed tomography (CT) scan, which was done 18 months after right extended hepatectomy and hepaticojejunostomy, revealed no definite evidence of somatostatin receptor (SSTR)-positive disease anywhere in the whole-body survey.

Hence, the authors concluded that biliary NETs are rare. However, in patients presenting with obstructive jaundice, biliary NETs should be kept in mind as a probable diagnosis, especially when typical risk factors for cholangiocarcinoma are not present.

Ref: Sherene H, Kasi V, Mahadevan GS, Rajasekaran S, Mehta SS. Unusual case of biliary neuroendocrine tumor. Case Rep Clin Radiol. 2025;3:35-8. doi: 10.25259/CRCR_104_2023