Primary lymphoepithelioma-like carcinoma of the lung. A clinicopathologic study of 11 cases

Abstract

Background. Lymphoepithelioma‐like carcinoma (LELC), best known to occur in the nasopharynx, can arise in a variety of sites, such as the salivary gland, thymus, lung, stomach, and skin. Primary LELC of the lung is very rare, with only limited information in the literature. Methods. The clinicopathologic features of 11 patients with pulmonary LELC collected from two regional hospitals in Hong Kong are described. Results. The patients, all Chinese, were aged 38 to 73 years (median, 54 years), with equal sex incidence. Two of the 8 patients were smokers. Four presented with coin lesions incidentally discovered on chest X‐ray, five with cough and blood‐stained sputum, and two with pleural effusion. The tumor formed a discrete (9 patients) or an ill‐defined (1 patient) nodule in the lung, or, rarely, showed extensive bilateral pulmonary involvement (1 patient). The major bronchi were not involved except in 1 patient. Three patients had lymph node metastasis at presentation; two of them had bone metastasis, one at presentation and one after 9 months. The tumors had pushing margins, and grew in the form of anastomosing islands and sheets, comprising syncytial‐appearing large cells with vesicular nuclei and prominent nucleoli. They were infiltrated by an appreciable number of small lymphocytes and plasma cells. Intratumoral amyloid globules were found in one tumor. In five patients, the tumor showed intraepithelial growth within the small bronchi; this could represent either the in‐situ phase of the tumor or pagetoid spread into the bronchial epithelium. The neoplastic cells of all patients harbored Epstein–Barr virus (EBV) as demonstrated by in situ hybridization for EBV‐encoded small nuclear RNAs. All eight Asian patients with pulmonary LELC previously reported in the literature similarly have been EBV‐positive, whereas the four reported Caucasian patients all have been EBV‐negative. Conclusion. Lymphoepithelioma‐like carcinoma of lung occurring in Asians is an EBV‐associated neoplasm; it also appears to occur at a higher frequency in Asians than Caucasians. It usually presents as a solitary sub‐pleural nodule, and there is no strong association with cigarette smoking. Most patients have early stage disease at presentation. From the limited available data, the behavior of LELC of lung is highly variable, ranging from apparent curability by excision (particularly for localized disease) to highly aggressive, extensive disease at presentation. Cancer 1995; 76:413–22.

Epstein–Barr virus-associated adenocarcinomas and squamous-cell lung carcinomas

“…7 Most of them are located in a peripheral situation. 6 All pulmonary lymphoepithelioma-like carcinomas reported in the literature showed a significant CD8 T-lymphocyte reaction corresponding with cytotoxic T-lymphocytes in the resting state. 24 In our series we describe five lymphoepithelioma-like cases with a similar lymphoid infiltrate that displayed characteristic vesicular nuclei.…”

“…A PCR-ELISA technique for human herpesvirus family detection was performed (Herplex t Genomica, SAU, Madrid, Spain) according to manufacturer's instructions. 20 Briefly, we extracted DNA from the microdissected 5-mm sections from paraffin blocks, and a PCR-ELISA 25 9 Lymphoepitelioma like 100% EBER, 44% LMP1, 0% EBNA2 158 NSCLCs 0% EBER +++ China 6 11 Lymphoepitelioma like 100% EBER +++ USA 12 80 Adenocarcinomas 0% EBER1 50 Mesotheliomas 0% EBER1 Japan 17 1 procedure was performed. The PCR mix contained primers against a 194 bp sequence of DNA viral polymerase from herpes simplex types 1 and 2, varicella zoster, cytomegalovirus, EBV and human herpesvirus 6.…”

“…It is defined as an undifferentiated carcinoma that shows a typical syncytial growth pattern of undifferentiated neoplastic epithelial cells, with vesicular nuclei and prominent nucleoli, similar to the nasopharynx counterpart. 5 Primary lymphoepithelioma-like carcinoma of the lung is very rare and most of reports come from Chinese or Taiwanese patients, [6][7][8] and to date the association of EBV with these kind of lung carcinomas is restricted to Asian patients 9,10 (Table 1).…”

The new World Health Organization classification of lung tumours

“…In addition, LCNEC comprise rare tumour types such as lymphoepithelioma-like carcinoma (EpsteinBarr virus-dependant epithelial proliferation) [37,38], clear cell carcinoma [39], and large cell carcinoma with rhabdoid phenotype [40]. Large cell carcinoma with rhabdoid phenotype as well as blastoma and desmoid round cell tumours, primitive neuroendocrine tumour (PNET) and paraganglioma belong to the category of non-neuroendocrine tumours that may display neuroendocrine markers.…”

Primary Pulmonary Lymphoepithelioma-like Carcinoma on FDG PET/CT

“…Early stage LELCs are observed on CT mostly as poorly circumscribed, peripherally placed <3.5 cm nodules and are not associated with lymphadenopathy [13,14]. On the other hand, determination of advanced stage disease is made by detection of large, well circumscribed, centrally located lesions with evidence of vascular encasement and peribronchovascular lymphadenopathy.…”