Intrahepatic Cholangiocarcinoma With Lymphoepithelioma-like Carcinoma Components Not Associated With Epstein-Barr Virus: Report of a Case

Abstract

A carcinoma displaying undifferentiated features with dense lymphoplasmacytic infiltration is defined as lymphoepithelioma-like carcinoma (LELC). Intrahepatic cholangiocarcinoma (ICC) with LELC components is rare, and most LELCs are associated with Epstein-Barr virus (EBV). We report here on a case of ICC with LELC components not associated with EBV. A 65-year-old woman was incidentally found to have a hepatic tumor in the caudate lobe. An extended right hepatectomy with lymphadenectomy was performed. Histologically, the tumor was mainly composed of large undifferentiated epithelial cells with vesicular nuclei, prominent nucleoli, indistinct cell borders, and heavy small lymphocytic infiltration, which are the characteristic features of LELC. Immunohistochemical studies revealed that the tumor cells were positive for cytokeratin 19 but were negative for glypican 3. In situ hybridization using EBV-encoded RNA was negative. Therefore, a diagnosis of ICC with LELC components not associated with EBV was made. Because there is limited information available regarding the prognosis and treatment of ICC with LELC components because of the limited number of reported cases, additional studies will be needed to clarify the clinicopathologic features of this disease.

Lymphoepithelioma-like intrahepatic cholangiocarcinoma with Epstein-Barr virus infection: report of a rare case

“…Hepatic LEL is extremely rare and usually found by accident. To the best of our knowledge, 67 cases of LEL-HCC, and 25 cases of LEL-ICC (Table 1) have been reported in the literature (2,(14)(15)(16)(17). A summary of the previously reported cases of LEL-ICC and the present case are summarized in Table 1.…”

“…LELC had a better prognosis than other neoplasms in the stomach and lungs (17). In patients diagnosed with LEL-ICC, the 5-year survival rate was 100% and 13.2% in classical cholangiocarcinomas (16).…”

“…Lee reported a patient with lymph node metastasis who underwent postoperative radiation and was still alive without recurrence 54 months after the surgery (15). A similar case was introduced by Aosasa et al and the patient was alive without recurrence more than 20 months after the surgery and post-operative radiation (17). Nevertheless, these promising results have still not been confirmed to be a result of post-operative radiation due to the commonly positive prognosis of LEL-ICC.…”

“…The LELC is considered to be strongly associated with EBV infection (17). Among the 26 cases, 19 (73.1%) patients were EBV positive, and only 3% of LEL-HCC was confirmed to be infected with EBV (2).…”

Thyroid-Like Intrahepatic Cholangiocarcinoma: Report of a Case and Review of the Literature

“…Multiple histologic variants have been described for ICC, including mucinous, lymphoepithelioma-like, signet ring cell, clear cell, adenosquamous, spindle cell, and rhabdoid-like carcinomas, as well as carcinomas with osteoclast-like giant cells and with papillary features. [4][5][6][7] Morphology with the appearance of a thyroid neoplasm is extremely unusual for ICC. 2,3 This unique feature has also been reported in carcinomas of the kidney, breast, and nasopharynx.…”