Sporadic EBV-associated lymphoepithelial salivary gland carcinoma with EBV-positive low-grade myoepithelial component

Salivary gland lymphoepithelial carcinomas (LECs) are associated with Epstein–Barr virus (EBV) in endemic areas, whereas sporadic cases are usually EBV negative. We have studied two EBV-associated LECs from Caucasian patients for their EBV gene expression profile and their immunophenotype. Tumour cells of case 1 showed expression of EBNA1 only, corresponding to an EBV latency type I. Tumour cells of this case expressed various basal and glandular cytokeratins. In case 2, the LEC was accompanied by a low-grade spindle cell lesion with an immunophenotype of myoepithelial cells, whereas the high-grade tumour expressed cytokeratin (Ck) 8 only. In case 2, the high-grade tumour showed an EBV lantency II pattern with expression of EBNA1, LMP1 and LMP2A (latency II). The spindle cell lesion of this case was also EBV-infected and showed low levels of EBNA1 and LMP1 expression, while LMP2A was not detectable. The detection of EBV in both components of case 2 together with immunophenotypic evidence of transition between both components supports the notion that at least some LECs arise through a low-grade myoepithelial intermediate. Expression of LMP2A may be of therapeutic interest because it may make such cases amenable to immunotherapy with EBV-specific cytotoxic T cells.