An EBV+ lymphoepithelioma-like cholangiocarcinoma in a young woman with chronic hepatitis B

Abstract

Epstein-Barr virus (EBV) is implicated in the tumorigenesis of a variety of malignancies, including Burkitt’s lymphoma, Hodgkin’s disease and nasopharyngeal carcinoma (NPC). EBV+ lymphoepithelioma-like cholangiocarcinoma (LELCC) is a rare type of intrahepatic cholangiocarcinoma with a distinct pathology and poorly understood treatment options. Morphologically, this neoplasm resembles undifferentiated NPC, a commonly EBV+ tumour with a prominent lymphoid infiltrate. Almost all of the current literature regarding LELCC describes early stage tumours that are treated surgically and achieve good outcomes. In contrast, this report documents a late stage LELCC treated unsuccessfully with systemic chemotherapy.

Lymphoepithelioma-like cholangiocarcinoma in middle-aged woman with Epstein-Barr Virus infection: a case report and review of the literature

“…Ling et al [10] retrospectively analyzed 40 cases of LELCC and found 80.0% of these cases were related to EBV. To date, only 29 cases of LELCC with Epstein-Barr Virus(EBV) Infection have been reported in the English literature [11], in addition, the diagnosis and differential diagnosis of LELCC are rarely reported [1,11]. The diagnosis and differential diagnosis of this extremely rare tumor are important for its identification.…”

“…6,Other less likely neoplasms were also ruled out using immunohistochemistry, including inflammatory myofibroblastic tumour (Anaplastic lymphoma kinase (ALK)), malignant melanoma (HMB45 and S100) and histiocytic sarcoma (CD163). These tumours can have large cells with a lymphocyte-rich tumour stroma, but EBV were negative in situ hybridization [11] .…”

“…The published literatures reported that majority of LELCC patients were discovered in the Asian countries [11,13,16]. LELCC with EBV infection tends to occur in middle-aged women with a female:male ratio of more than 3:1, the range of age was 19 to 71 years (mean age, 53.39 years) [8][9][10][11][12]. No preference in the tumor location was found between the left and the right hepatic lobe [17].…”

“…Zhang et al [26]found that Only 8 cases had recurrence in 52 cases of LELCC reported by searching English literature. Gearty et al [11] retrospectively analyzed the 29 LELCC cases with EBV infection, found that 8 patients died of the disease. Most cases were early stage tumours treated with surgical resection, including one successfully resected lymph node metastasis.…”

“…Microscopically, the boundary of the tumor is clear, and there is an incomplete fibrous capsule around the tumor, Small tumor cells were seen outside the capsule.The tumors with varied glandular differentiation were adenocarcinomas, which were characterized by tubular, trabecular, or cord-like patterns with variable degrees of differentiation tumor cells were cubic, large in size, rich in cytoplasm and eosinophilic The nuclei was round or oval with an irregular nuclear membrane vesicular nuclei, prominent nucleoli, and a syncytial cytoplasmic appearance, rare mitoticfigures.There are extensive lymphoplasmacytic infiltration in the stroma, Epithelioid granulomas and numerous lymphoid follicles could be seen. Occasionally, obvious granulomatous reactions and many multinucleated giant cells coexisted[11] .…”