Epstein-Barr Virus-Associated Lymphoepithelioma-Like Intrahepatic Cholangiocarcinoma: A Case Study and Literature Review
Year: 2022
Type: article
Study design: Case Report
Abstract: Lymphoepithelioma-like intrahepatic cholangiocarcinoma (LELCC) is a genetically distinct, rare subtype of intrahepatic cholangiocarcinoma. LELCCs have a strong association with Epstein-Barr virus (EBV), though the EBV associated LELCC (EBV+LELCC) carcinogenesis pathway has not been fully delineated. Only 38 cases of EBV+LELCC have been reported in the literature to date, most commonly occurring in females and Asians. Only 2 of the 38 cases are in patients with inflammatory bowel disease. In this case study, we report two cases of EBV+LELCC in male patients with inflammatory bowel disease. The first patient is a 29 year old Bangladeshi male with ulcerative colitis and primary sclerosing cholangitis. He was initially diagnosed with an intrahepatic lesion on imaging that was confirmed EBV+LELCC by biopsy. He underwent surgical resection and adjuvant chemotherapy. He is recurrence free two years after surgery. The second patient is a 70 year old American male with ulcerative colitis. He presented with fevers of unknown origin and was found to have new biliary strictures on imaging. Subsequent ERCP brushings were positive for adenocarcinoma. Patient underwent surgical resection and will undergo adjuvant chemotherapy and chemoradiation; final pathology revealed EBV+LELCC. Most published cases of patients with EBV+LELCC report increased survival when compared to patients with conventional intrahepatic cholangiocarcinoma (C-ICC); the mechanism for this observed difference, however, has yet to be elucidated. Further research is needed to characterize the clinicopathologic features of EBV+LELCC to improve outcomes for patients with this rare disease and provide insight into potential therapeutic targets for the treatment of C-ICC.
Source: HPB
Institution: Mayo Clinic
Language: English
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $3,300
OpenAlex ID: W4312256051