Primary lymphoepithelioma-like carcinoma of the lung: An unusual cancer and clinical outcomes of 14 patients

Abstract

Advanced lung cancer is considered to exhibit a poor prognosis; however, the pulmonary lymphoepithelioma-like carcinoma (LELC), a rare subtype of non-small cell lung cancer (NSCLC), exhibits an improved prognosis, compared with non-LELC. The present study aimed at investigating the clinical manifestation, imaging characteristics, pathology, tumor markers, treatment and prognosis of primary LELC of the lung. A total of 14 patients with pulmonary LELC were confirmed by surgery and pathology. Clinical data of those patients were retrospectively reviewed including age, sex, smoking history, symptoms, computed tomography (CT) results, Epstein-Barr virus-encoded RNA (EBER) status, treatment and outcomes. In the present study, there were 7 males and 7 females who ranged in age between 22 and 64 years (mean, 51.21±11.37 years) and who all were from eastern China. The tumor-node-metastasis stage ranged between stages I and IV, with 71.43% of the patients at advanced stage (stages III and IV). The results of the present study identified 100% positive expression of EBER. Tumors located centrally were of significantly increased size, compared with peripheral tumors (P<0.05), and lymphadenopathy was more common in patients with advanced stage (P<0.05). The majority of patients were treated with surgery, platinum-based chemotherapy or radiotherapy. At time of writing, 12 patients were alive and the longest survival time was 60 months. Pulmonary LELC typically affected young patients and was not associated with smoking history; however, pulmonary LELC was associated with Epstein-Barr virus infection in the Asian population. The majority of patients were in early or locally advanced stages and exhibit an improved prognosis compared with other types of NSCLC. Pulmonary LELC was sensitive to chemotherapy and surgery, with postoperative chemotherapy-based multimodality treatment recommended.

Keywords: Epstein-Barr virus; lymphoepithelioma-like carcinoma; prognosis; treatment.

Figures

Figure 1.

CT scan of a 52-year-old female with stage IV primary pulmonary LELC. Arrow A, a CT scan revealed a large and well-defined soft tissue mass located in the right lower hilus of the lung (size, 6.5×5.0 cm). Arrow B, the bronchus in right lower lobe was narrowing with a thickened wall. Arrow C, multiple masses or nodules were observed in the right side of the chest wall, pleura and right oblique fissure with variable sizes (largest, ~1.4×2.7 cm). Arrow D, a limited amount of effusion was observed in the right side of the chest. CT, computed tomography; LELC, lymphoepithelioma-like carcinoma. HFS, head first-supine; MA, milliampere; KV, kilovolt; L, windows level; W, windows width; [L], length; GT, gradient; TI, time.

Figure 2.

CT scan of a 56-year-old male with stage IIIA primary pulmonary LELC. Arrow A, a CT scan identified a rough-edged, lobular and spiculated nodule in the apicoposterior segmental bronchus of the left upper lobe (size, 1.6×1.2 cm). Arrow B, pleural indentation was also observed. CT, computed tomography; LELC, lymphoepithelioma-like carcinoma. FFS, feet first-supine; MA, milliampere; KV, kilovolt; L, windows level; W, windows width; [L], length; GT, gradient; TI, time.

Figure 3.

CT scan of a 61-year-old male with stage IIIA primary pulmonary LELC. The CT scan revealed a rough-edged nodule in the lingular segment of the left upper lobe with cavity inside the lesion. CT, computed tomography; LELC, lymphoepithelioma-like carcinoma. FFS, feet first-supine; MA, milliampere; KV, kilovolt; L, windows level; W, windows width; [L], length; GT, gradient; TI, time.

Figure 4.

PET-CT scan of a 38-year-old male with stage IIIB primary pulmonary LELC. Arrow A, a PET-CT scan identified a highly metabolic soft tissue (size, 3.5×3.4 cm) with slightly rough edge, lobular, spiculated, inhomogeneous density and pleural indentation in the anterior segment of the right upper lobe. Radioactive uptake increased and the SUVmax was 5.4. Arrow B, multiple enlarged lymph nodes in the right-side mediastinum and right hilus of the lung. The largest lymph node was located in the mediastinum (size, 2.4×2.2 cm), part integrated into clumps and radioactive uptake was slightly increased (SUVmax, 3.5). PET-CT, positron emission tomography-computed tomography; LELC, lymphoepithelioma-like carcinoma; SUVmax, maximum standardized uptake value. KV, kilovolt; MA, milliampere; MAS, milliampere sec; L, windows level; W, windows width; [L], length.