Objective To investigate the clinicopathologic features, diagnosis, treatment and prognosis of the primary lymphoepithelioma-like carcinoma (PLELC) of the lung. Methods Clinical and pathological data of one patient with PLELC of the lung were reviewed. PLELC of the lung was analyzed through review of literatures. Results A mass in the upper lobe of the left lung and atelectasis were detected by CT in a 36 year old nonsmoker male. He presented with cough, expectoration, fever and shortness of breath for one month. Neoplasm in left main bronchus was observed through bronchoscopy. Histological study showed cohesive clusters of comprising syncytial-appearing cells with vesicular nuclei and tumor infiltrating lymphocytes. Immunohistochemistry showed positive expression of Epstein-Barr virus (EBV) encoded small nuclear RNA. Nasal CT scan was negative for suspicious nasopharyngeal mucosal lesion or tumor. Then he was diagnosed as PLELC. The patient received docetaxel+nedaplatin regimens for 6 cycles with stable disease, but one month later after the end of chemotherapy, he was readmitted to hospital for dyspnea. CT scans showed progression of the lesion with massive pericardial effusion and pleural effusion. The patient could not tolerate chemotherapy, then received supportive treatment and died soon. Totally 18 Chinese articles and 25 foreign articles were screened out. Literature review suggested that patients with PLELC of the lung usually occurred in young nonsmokers and without gender difference. PLELC of the lung was closely associated with EBV infection. Its clinical manifestations were not specific, so that histopathological and immunohistochemical analyses were the main method of diagnosis. PLELC of the lung may respond to chemotherapy and radiotherapy. Surgery-based multimodality treatment was recommended. Patients with early or resectable disease had better prognosis, but patients with unresectable disease had poor prognosis. Conclusions PLELC of the lung may be closely associated with EBV infection. Histopathological and immunohistochemical analysis is the main method of diagnosis. Surgery-based multimodality treatment should be recommended because of its high response to chemotherapy and radiotherapy. Patients with early or resectable disease have better prognosis.
Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is a rare type of lung cancer with special characteristics of epidemiology, clinical diagnosis, treatment and prognosis. It has close relationship with Epstein-Barr virus (EBV) infection and has prominent regional feature. Most patients are young and non-smoking. There is no specificity of clinical manifestation. Most patients are asymptomatic at the time of diagnosis. As for treatment, the standard treatment for early stage disease is complete resection. Platinum-based doublet chemotherapy has been applied in locally advanced disease as the first line therapy. Due to its rarity, the treatment of advanced PPLELC is still lack of evidence of large sample randomized controlled trial. Whether target therapy or immunotherapy is effective is worth further study. This article reviews the research progress of PPLELC, to give a particular sight for clinicians and provides a better understanding of this rare tumor for researchers.
Objective To investigate the clinicopathological features, treatment and prognosis of primary pulmonary lymphoepithelioma-like carcinoma (PPLELC). Methods Forty-five patients with PPLELC admitted to Sichuan Cancer Hospital from April 2016 to March 2023 were retrospectively analyzed. Their clinical data and survival were collected and summarized. Results Of the 45 patients, 28.9% (13/45) were asymptomatic, 97.2% (35/36) were positive for Epstein-Barr virus encoded small RNA, 83.3% (10/12) were positive for programmed cell death-ligand 1 (PD-L1), and the tumor proportion score (TPS) fluctuated between 10.0% to 95.0%. Genetic testing revealed 1 case of EGFR gene 19-Del mutation. Among the 45 patients, 28 cases received surgical treatment, of which 16 received adjuvant chemotherapy, 8 received adjuvant radiotherapy, and 2 received neoadjuvant chemotherapy. As of the follow-up cut-off date, 33 patients survived and 12 patients died, with a median survival time not reached and an overall survival rate of 70.2% and 63.2% at 3 and 5 years, respectively. The 3- and 5-year disease-free survival rates were 67.2% and 57.6% for the 28 operated patients, respectively, and the progression-free survival rates were 58.3% and 29.2% for the 13 stage Ⅳ patients, respectively. Conclusions PPLELC is associated with Epstein-Barr virus infection and is a rare subtype of non-small cell lung cancer (NSCLC). Most patients do not have common gene mutations such as EGFR or ALK and have a high rate of positive PD-L1 expression. The disease is usually treated with a multi-method combination of mainly surgery, and is sensitive to radiotherapy and chemotherapy and has a better prognosis than other types of NSCLC.