lymphoepithelial carcinoma ( lec ) of salivary gland is a rare neoplasm with a striking geographic and ethnic distribution with a vast majority occurring in north americans , greenland eskimos and asian orientals . the parotid gland is involved in more than 90% of tumors , with the submandibular gland being an uncommon site . a high proportion of these tumors show consistent association with epstein - barr virus ( ebv ) . although a variant of undifferentiated carcinoma , the prognosis seems to be better than other undifferentiated carcinomas of salivary gland , probably because lymphoid stroma has a role in limiting the aggressiveness of this lesion . a 13 year old male presented with a hard , fixed 4  4-cm mass located in the right submandibular region . the aspiration yielded highly cellular smears comprising markedly atypical epithelial cells disposed in cohesive clusters and few small groups . an intimate intermingling of mature lymphocytes within these epithelial clusters was seen . a diagnosis of metastatic poorly differentiated carcinoma was suggested . the lymph node chain consisted of eight enlarged lymph nodes , three of which had a grey - white cut surface . the microscopic examination disclosed a malignant neoplasm characterized by anastomosing cords , syncytial nests and islands of highly undifferentiated cells with indistinct borders , eosinophilic cytoplasm , large vesicular nuclei and single to multiple prominent nucleoli [ figure 2a ] . the epithelial component was sharply demarcated from stroma consisting of diffuse sheets and aggregates of lymphoid cells forming germinal centres at few places as well as fibrosis . a diagnosis of lec was made with a suggestion to rule out metastatic nasopharyngeal carcinoma . a detailed otorhinolaryngological examination was undertaken with inclusion of multiple site biopsies which were negative for malignancy . immunohistochemistry ( ihc ) demonstrated ck - positive and s-100-negative tumor cells intermingled with cd45-positive lymphoid cells [ figure 2b , c ] thereby , clinching the diagnosis of primary lec of submandibular gland . highly cellular smears showing cohesive aggregates of atypical epithelial cells lying against a background of mature lymphocytes ( h and e , x100 ) . inset shows atypical cells having indistinct cytoplasmic borders , large vesicular nucleus with single to multiple prominent nucleoli and finely granular chromatin ( h and e , 1000 ) ( a ) histopathological section of lec showing nests and islands of tumor cells embedded in a densely lymphoid and fibrotic stroma ( h and e , 400 ) ; ( b ) pan - ck immunostain highlighting tumor cells ( ihc , 1000 ) ; ( c ) cd45 decorating the interspersed lymphoid cells ( ihc , 1000 ) lec is an unusual type of squamous cell carcinoma predominantly occurring in the upper aero - digestive tract . primary lec occurring in the salivary gland is extremely rare accounting for 0.4 - 1.0% of the epithelial tumors of the salivary gland . this lesion displays a mysteriously strong racial predilection with the age at presentation ranging from 20 to 60 years and female to male ratio of 1.5:1 . the parotid gland is the favored site for lec of salivary gland constituting more than 90% of lesions . the ratio of lec of the parotid gland to submandibular gland in accumulated cases in literature is 17:1 . although important pathogenetically , ebv does not seem to influence the prognosis of these lesions . most lec develop de novo with only a subset of cases arising from benign lymphoepithelial lesion ( blel ) . hilderman et al described a malignant lesion of parotid gland that resembled blel and called it malignant lymphoepithelial lesion ( mlel). this obfuscatory term was replaced by  lymphoepithelial carcinoma ( lec) after the squamous epithelial origin was confirmed by electron microscopic studies of kott et al . although medical literature is replete with case reports describing histopathological features of lec , only a handful of case reports describing the cytopathological features of this lesion the earliest description of cytological features of this entity was made by gunhan et al . and thompson et al . in parotid lesions . characteristically , the smears are cellular comprising biphasic population of cells with one component of poorly differentiated epithelial cells exhibiting marked pleomorphism , high nucleocytoplasmic ratio , vesicular nuclei with finely granular chromatin and prominent nucleoli . the other component consists of mature lymphocytes with occasional macrophages . before establishing a diagnosis of primary lec of the salivary gland , it is essential to exclude metastasis from an undifferentiated carcinoma . anatomically , nasopharyngeal lymphoepithelioma is the most likely candidate to metastasize to this area . according to gunhan et al . , the cytological proof of malignancy in cases of primary or metastatic lec is generally easy but , specifying the type of lesion as lec may be difficult . the characteristic cytological features of cohesive large epithelial cell clusters intermingling with mature lymphocytes , histiocytes and plasma cells seen in lec , are similar to those seen in nasopharyngeal carcinoma . histopathologically , the presence of blel adjacent to the tumor is helpful but , multiple site biopsies from the upper aerodigestive tract are mandatory to rule out nasopharyngeal carcinoma . the cytological differential diagnosis for nasopharyngeal lymphoepithelioma are also valid for lec of salivary gland and include large cell lymphoma and poorly differentiated carcinoma of various organs . differentiation from large cell lymphocytic and histiocytic neoplasm is helped by ihc markers like cd20 , cd45ro , cd68 , leu-1 , ki-67 and epithelial markers . another differential diagnosis is amelanotic melanoma where marker proteins like anticytokeratins , anti - s100 and anti - hmb-45 help . in the present case although most case reports describe the cytological smears of lec as moderately to highly cellular , in the present case the lesion remained masked despite two fna attempts due to scant cellularity . have also made a brief mention regarding the sparse cellularity of smears consisting of atypical cells intermingled with lymphocytes . thus , the lesion may remain cryptic and the cytopathologist must not refrain from a repeat fna or biopsy if the clinical suspicion is high . although a rare entity , lec must be considered in the differential diagnosis when a cytopathologist is confronted with aspirates comprising atypical epithelial cells admixed with lymphocytes . the lesion may remain masked under the garb of reactive lymphadenitis or due to scant cellularity , warranting repeat aspiration / biopsy if the clinical suspicion is high . the presence of large undifferentiated cells with prominent nucleoli and fine chromatin , admixed with lymphocytes in cytology aspirates are fairly distinctive to at least suggest a possibility of lec .
a case of lymphoepithelial carcinoma ( lec ) occurring in right submandibular gland of a 13-year - old indian male is presented , wherein the lesion unveiled itself only after multiple fine needle aspiration ( fna ) procedures . 
 this unusual neoplasm has high frequency of occurrence in eskimos and a predilection for the parotid gland . 
 the aspirates obtained were highly cellular comprising tight clusters of atypical epithelial cells with admixture of lymphocytes . 
 histopathological examination of the resected submandibular gland and lymph node chain was consistent with the diagnosis of lec . 
 immunohistochemistry ( ihc ) revealed cytokeratin ( ck)-positive and s-100-negative tumor cells lying admixed with cd45-positive lymphoid cells . 
 a detailed otorhinolaryngological examination with inclusion of multiple biopsies was found negative for any primary tumor . 
 although histopathological features of this entity are well established , only a handful of case reports describing cytological features of this entity are present in medical literature . 
 we conclude that the cytomorphological features of lec are sufficiently distinctive to at least , suggest a possibility of this lesion .