Presentation
Two-week history of the right upper quadrant (RUQ) abdominal pain, nausea, loss of appetite, and a 3-month history of weight loss. Biliary stenting during the hospital course.
Patient Data
There is a large central hepatic mass extending from the porta hepatis to the liver dome with associated intrahepatic biliary dilatation, and numerous intrahepatic satellite lesions/metastases.
A palliative biliary stent going through the mass is seen.
The Gallbladder neck is inseparable from the dominant hepatic mass.
There is focal thickening at the fundus of the gallbladder with linear calcifications may represent adenomyomatosis.
There is diffuse mural thickening of the duodenum indicating a diffuse tumoral process.
There is visible thrombosis of the superior mesenteric vein with extensive thrombosis of the main portal vein and with thrombus distally extending to the right and left portal vein.
There is also a large left para-aortic lymph node representing retroperitoneal adenopathy and likely lymph node metastasis.
Case Discussion
This patient presented with an acute biliary obstruction and had two emergency biliary stents placed. Biliary biopsies were taken during the procedure with the following findings:
intrahepatic biliary biopsy: moderately to poorly differentiated adenocarcinoma with some biliary differentiation
periportal lymph node biopsy: moderately to poorly differentiated adenocarcinoma with extensive necrosis
biliary hilum biopsy: highly atypical cells which are suspicious of malignancy
Based on imaging and biopsy results, the patient was confirmed to have a mass-forming intrahepatic cholangiocarcinoma. This patient lacked risk factors commonly associated such as heavy drinking, smoking, IBD, or obesity. Given the advanced stage and presence of metastasis on CT, the prognosis is poor.
Intrahepatic cholangiocarcinoma is a rare and aggressive malignancy that presents with nonspecific symptoms and image findings, often leading to late-stage diagnosis 1. Jaundice and scleral icterus are commonly observed secondarily to biliary obstruction and indicate a more advanced stage and worse prognosis 1,5. The mass-forming subtype, as observed in this case, presents as a distinct hepatic mass occurring in about 20% of cholangiocarcinomas 3.
For small to less advanced tumors, surgery might be an option. Given the advanced stage of the disease, curative surgical options are not feasible in this patient 1. Emergency palliative biliary stenting was performed to relieve the biliary obstruction. Palliative chemotherapy and radiotherapy may be considered to slow disease progression 1. In this case, the patient refused and agreed to palliative and hospice care.
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