Primary Lymphoepithelioma-like Carcinoma of the Lung in Singapore—A Rare Malignancy

Abstract Background: Primary lymphoepithelioma-like carcinoma (LELC) of the lung is a rare histological type of lung cancer with a relatively better prognosis. It is common in Chinese population, is associated with Epstein Barr Virus (EBV), and has morphological similarities to nasopharyngeal carcinoma. Methods: We retrospectively analysed medical records of patients with LELC at Tan Tock Seng Hospital, Singapore from November 2010 to January 2016 and evaluated baseline characteristics, staging, therapeutic interventions and survival. Results: Ten patients were diagnosed with pulmonary LELC, all were Chinese. The median age (range) at diagnosis was 62 (42-88) years, with a female preponderance ratio of 4:1. All had Epstein-Barr virus encoded small non-polyadenylated ribonucleic acid positive status. Six (60%) patients had stage IV, 3 (30%) stage IIIA, and 1 (10%) stage IIA disease. The median overall survival was 24.2 (4.7-59.4) months; in stage II, IIIA, and IV it was 27.6, 20.7 (19.8-48.9), and 26.6 (4.7-59.4) months respectively. The 1-year, 2-year, 3-year, and 5-year survival rate was 90%, 50%, 30%, and 10% in the whole group, but 100%, 43%, 28.6%, and 14.3% in 7 patients who received chemotherapy. Gemcitabine/carboplatin was the most commonly used 1st line chemotherapy. Conclusion: LELC is associated with Chinese ethnicity, younger age of onset, EBV, lack of smoking, advanced stage, and better prognosis than other subtypes of lung cancers. For improving outcomes, future efforts should focus on increased awareness, early diagnosis, development of an ASEAN registry and international collaboration. Options such as EBV-directed adopted immunotherapy or the role of EBV vaccine also merit evaluation.