Lymphoepithelioma-like carcinoma in the setting of cholangiocarcinoma in the absence of underlying Epstein-Barr virus is an incredibly rare entity. However, all of the reports that exist, which are few and far between, describe this vignette in patients of Far Eastern origin.We present the first case report of Epstein-Barr virus-negative lymphoepithelioma-like carcinoma-associated cholangiocarcinoma occurring in a patient of European descent. The 62-year-old woman described here presented with nonspecific symptoms of lethargy, low-grade pyrexia, and raised white blood cell count. Subsequent investigations revealed a large hepatic mass with subsequent hepatectomy yielding a large tumor containing poorly differentiated adenosquamous cholangiocarcinoma alongside a rich infiltrate of lymphocytes and lymphoplasmacytic inflammatory infiltrate. Epstein-Barr virus in situ hybridization was negative in both the malignancy and surrounding tissues. Reflecting its rarity, the pathogenesis of this specific malignancy is poorly understood, as also are the treatment and prognosis.
- Epstein-Barr virus