Background: Lymphoepithelioma-like carcinoma (LELC) of the lung is a rare subtype of non-small-cell lung cancer with no established treatment guidelines. We report a case of primary pulmonary LELC (PPLELC) in a young, non-smoking woman with a good response to two types of immune checkpoint inhibitors (ICIs).
Result: A 39-year-old female was diagnosed with metastatic EBV-related PPLELC. The initial PET-CT scan showed a 5.5 cm hypermetabolic lesion at the right middle lobe with intrapulmonary metastases. A transbronchial biopsy revealed a non-keratinizing carcinoma with positive staining for AE1/AE3, p40, and p63. EBER was positive on in-situ hybridization. ENT examination was unremarkable. Molecular analyses of the tumor showed no EGFR, ALK, ROS, BRAF, or KRAS gene mutations, microsatellite stable, and low tumor mutational burden. PD-L1 expression (TPS) was 10%. The patient was initially treated with carboplatin and gemcitabine for 6 cycles, with a partial response after 3 cycles. The PET-CT scan following 6 cycles showed tumor progression. She was then treated with pembrolizumab. The scan after 3 cycles showed stable disease (SD). The treatment was continued until 12 cycles had been completed. The patient subsequently had disease progression. She received docetaxel for 6 cycles, with the best response being SD. Again, the patient demonstrated disease progression in her lungs and liver. She received 3 cycles of capecitabine followed by 2 cycles of pemetrexed with no response. The next line of treatment consisted of atezolizumab/bevacizumab/carboplatin/paclitaxel (as inIMpower150). Unexpectedly, she tolerated this regimen quite well. The scan after 3 cycles showed SD. However, her disease progressed shortly after 6 cycles. Oral vinorelbine was prescribed, and, again, there was no response. She passed away in June 2020 with an overall survival of 28 months from the initial diagnosis.
Conclusion: Until now, no mutation has been reported in EBV-related PPLELC. In this case, the patient responded to anti-PD-L1 after having a progression on anti-PD1. Therefore, ICIs may be beneficial in this rare subtype of lung cancer with PD-L1 expression and no actionable gene mutations.