title: Primary pulmonary lymphoepithelioma-like carcinoma: fifty-two patients with long-term follow-up.
abstract: BACKGROUND
  Pulmonary lymphoepithelioma-like carcinoma (LELC) is a rare kind of cancer.
  
  
  METHODS
  In this study, the authors evaluated 52 patients with pulmonary LELC who had long-term follow-up. Clinical characteristics, tumor markers, epidermal growth factor receptor (EGFR) mutation status, treatments, and outcomes were analyzed.
  
  
  RESULTS
  Pulmonary LELC mostly affected young, nonsmoking patients. Most patients were in early or locally advanced stages and received multimodality treatment. Serum levels of neuron-specific enolase and cytokeratin 19 fragment 21-1 were elevated in 11 of 20 patients and 10 of 16 patients, respectively. Mutational analysis of EGFR was done in 11 patients, and all were wild type. The median overall survival (OS) for all the patients was not reached, and the 2-year and 5-year OS rate was 88% and 62%, respectively. The patients with early tumor stage, normal serum lactate dehydrogenase level, normal serum albumin level, without lymph node metastasis, and those who underwent complete resection had significantly better OS (P < .05); and the serum albumin level was an independent prognostic factor in a Cox regression model (P = .005). For all patients who underwent complete resection, whether or not they received adjuvant chemotherapy did not affect OS (P > .05); whereas, for patients with stage IIIA disease who underwent complete resection, adjuvant chemotherapy was correlated with a significantly better prognosis (P < .05).
  
  
  CONCLUSIONS
  Pulmonary LELC obviously is a distinct entity of lung cancer that has a better prognosis, because patients with LELC can receive multimodality treatment, and LELC has biologic behavior similar to that of nasopharyngeal carcinoma. The current results indicated that future collaborative efforts are needed to determine the optimal treatment methods for this uncommon malignancy.
authors.affiliation: Department of Medical Oncology, Cancer Center, Sun Yat-sen University, Guangzhou, Guangdong, People's Republic of China.
authors.forename: Ying
authors.initials: Y
authors.lastname: Liang
authors.forename: Liang
authors.initials: L
authors.lastname: Wang
authors.forename: Yujia
authors.initials: Y
authors.lastname: Zhu
authors.forename: Yongbin
authors.initials: Y
authors.lastname: Lin
authors.forename: Hui
authors.initials: H
authors.lastname: Liu
authors.forename: Huilan
authors.initials: H
authors.lastname: Rao
authors.forename: Guangchuan
authors.initials: G
authors.lastname: Xu
authors.forename: Tiehua
authors.initials: T
authors.lastname: Rong
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000235:genetics*; D002289:Carcinoma, Non-Small-Cell Lung / Q000097:blood / Q000235:genetics / Q000401:mortality* / Q000473:pathology / Q000628:therapy* / Q000821:virology; D017024:Chemotherapy, Adjuvant; D002648:Child; D004252:DNA Mutational Analysis; D020031:Epstein-Barr Virus Infections / Q000150:complications; D066246:ErbB Receptors / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D053208:Kaplan-Meier Estimate; D008175:Lung Neoplasms / Q000097:blood / Q000235:genetics / Q000401:mortality* / Q000473:pathology / Q000628:therapy* / Q000821:virology; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D010166:Palliative Care / Q000379:methods; D011013:Pneumonectomy; D011379:Prognosis; D035583:Rare Diseases; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor; D066246:ErbB Receptors