Lymphoepithelioma-like Carcinoma of Rectum: Possible Relation with EBV

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Summary

Lymphoepithelioma-like carcinoma (LEC) of the colon is very rare. Here we report a case of LEC originating in the rectum that was closely associated with Epstein-Barr virus (EBV) infection. The histologic and immunohistologic features, namely, poorly differentiated adenocarcinoma with lymphoid stroma, showed this tumor to be an LEC. The EBV genome was detected by PCR using DNA obtained from tumor tissue sections. Immunohistochemically, EBV-determined nuclear antigen 2 was detected in the tumor cells, and in situ hybridization using EBV-encoded small RNAs probe showed positive labeling in some tumor cells together with a few stromal lymphoid cells. There are some reports of LEC cases that originated in the colon; however, a relation with EBV was not demonstrated. We report here a case of LEC of the rectum demonstrating a possible relation with EBV.

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      These tumors are associated with microsatellite instability (MSI). Major studies on medullary carcinomas of the gastrointestinal tract concluded that these tumors have an organoid, solid, sheet-like or trabecular pattern with peri and intratumoral lymphocytes and consist of round to polygonal cells with high nuclear/cytoplasmic ratios and rounded nuclei with small central nucleoli.5,6 LECs and LELCs are made up of small clusters and aggregates of tumor that are broken up by large numbers of intratumoral lymphocytes.

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      The term LEC was first used by Hilderman in 1962 [11], and it denotes as a variant of nonkeratinizing undifferentiated carcinoma that is associated with a characteristic dense lymphoid stroma. Apart from NP, where it is most commonly seen, LEC has been described in a whole range of organs such as salivary glands, thymus, stomach, lung, skin, larynx, floor of the mouth, uterine cervix, tonsil, sinonasal tract, lacrimal gland, breast, extrahepatic bile duct, pancreas, renal pelvis, thyroid, ovary, urinary bladder, prostate, Bartholin gland, ureter, mandible, vagina, rectum, trachea, and kidney [1,3,12-27]. Especially in endemic areas, EBV is strongly associated with LEC occurring in most sites, and there is virtually a 100% association with LEC (nonkeratinizing; differentiated and undifferentiated subtypes) occurring in the NP and salivary glands [2-4].

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