Histopathological, Molecular and Clinical Profiling of Lymphoepithelioma-Like Carcinoma of the Bladder (LELC-B).

Lymphoepithelioma-like urothelial carcinoma of the urinary bladder (LELC-B) is a rare histologic subtype characterized by strong immune cell infiltrates. A better prognosis and favorable response rates to immune-checkpoint inhibitors (ICI) have been described. We aimed to characterize the molecular profiles and immune cell infiltration of LELC-B for a better understanding and its therapeutic implications. We identified eleven muscle-invasive bladder cancer cases with pure and mixed LELC-B. PD-L1 expression and mismatch-repair (MMR) proteins were evaluated using immunohistochemistry. We calculated the tumor-mutational burden (TMB) and characterized mutational profiles using whole exome DNA-sequencing data. Transcriptomic signatures were detected using the NanoString nCounter PanCancer IO360 panel. Multiplex immunofluorescence of tumor microenvironment (PD-L1, PanCK, aSMA, Vimentin, CD45, Ki67) and T-cells (CD4, CD3, PD-1, CD163, CD8, FoxP3) was used to quantify cell populations. All LELC-B cases were highly positive for PD-L1 (median TPS/TC 70%; range 20-100; median CPS 100; range 50-100), MMR-proficient and negative for Epstein-Barr virus infection. Immune cell infiltrates were characterized by high CD8+ T-cell count and high PD-1/PD-L1 expression on immune and tumor cells. LELC-B showed upregulation of signaling pathways involved in immune cell response. Most common mutations were found in chromatin remodeling genes causing epigenetic dysregulation. All LELC-B cases showed high TMB of 39 Mut/Mb (IQR 29-66). In conclusion, LELC-B is a highly immunogenic tumor, showing strong upregulation of PD1/PD-L1 and making ICI a promising treatment option.

Prognostic significance of circulating Epstein-Barr virus DNA in pulmonary lymphoepithelioma-like carcinoma: A meta-analysis and validation study.

In small-scale studies, circulating Epstein-Barr virus (EBV) DNA levels have prognostic value in patients with pulmonary lymphoepithelioma-like carcinoma (LELC). Therefore, we performed a comprehensive meta-analysis to evaluate the prognostic significance of circulating EBV DNA levels in patients with pulmonary LELC. Studies that discussed the prognostic significance of circulating EBV DNA detection in pulmonary LELC were eligible for inclusion in this study. The overall survival (OS) and progression-free survival (PFS) were the primary outcomes. Pooled hazard ratio (HR), 95% confidence intervals (CIs), and p value were calculated to estimate the prognostic significance of EBV DNA levels. Additionally, we conducted a further observation using an independent cohort. The pooled HR and 95% CI of pretreatment EBV DNA levels for OS and PFS were 3.63 (95% CI: 2.90-4.55) and 2.88 (95% CI: 1.90-4.38), respectively. The pooled HR and 95% CI for Posttreatment EBV DNA levels for OS and PFS were 3.77 (95% CI: 2.96-4.80) and 3.52 (95% CI: 1.91-6.51, p < 0.001), respectively. The independent cohort showed similar results that patients with high pretreatment EBV DNA or positive posttreatment EBV DNA had significantly inferior PFS. Circulating EBV DNA levels provide prognostic values of survival and treatment response in pulmonary LELC patients.

Epidemiology and prognosis of lymphoepithelioma-like carcinoma: a comprehensive analysis of surveillance, epidemiology, and end results (SEER) database.

AIM: Lymphoepithelioma-like carcinoma (LELC) is a rare histological types of solid tumors. The present study aims to comprehensively describe the demographic and clinical features of LELC using surveillance, epidemiology, and end results (SEER) database, with an emphasis on the prognostic difference according to primary tumor sites of LELC. MATERIALS AND METHODS: A population cohort with histologically diagnosed LELC were identified from SEER database between 1973 and 2016. Age-adjusted incidence rates and cancer-specific survival (CSS) were determined. Cox-regression proportional hazards model was used for both univariate and multivariate analyses. RESULTS: In total, 2106 patients with LELC were identified, with the most common diagnosed primary tumor site of nasopharyngeal LELC (56.22%), followed by non-nasopharyngeal head and neck LELC (21.32%) and respiratory system (7.83%). The overall age-adjusted incidence of LELC was 0.091 per 100,000. The CSS rates of LELC patients at 5, 10, 15, and 20 years were 76%, 69%, 65%, and 61%, respectively. A tendency of decreasing incidence of LELC was observed in the past decade. Univariate analysis indicated that sex [hazard ratio (HR) 1.21, p = 0.031], year of diagnosis (HR 0.60 and 0.63, p < 0.001), race (HR 1.29, p = 0.0021), age (HR 1.25, p = 0.0072), summary tumor stage (HR 1.97, and 4.57, both p < 0.001), number of positive LN(HR2.21, p < 0.001), surgery (HR 0.58, p = 0.0033), chemotherapy (HR 1.19, p = 0.037) and primary tumor site (p < 0.001) were significant factors associated with prognosis of LELC. In multivariate analysis, age (HR 1.75, p = 0.03), distant stage (HR 6.57, p = 0.0001), number of positive LN (HR 2.63, p = 0.0015) and non-nasopharyngeal head and neck LELC (HR 0.37, p = 0.0031) were significantly independent predictors for CSS of LELC. In sub-group analysis, radiotherapy significantly improves CSS for nasopharyngeal LELC (HR 0.57, p = 0.0002), while surgery significantly improve CSS for non-nasopharyngeal LELC (HR 0.33, p < 0.0001). CONCLUSION: Based on SEER data analysis, age older than 50 years, distant stage and more than three positive LN are significantly associated with worse CSS for LELC, while the prognosis of non-nasopharyngeal head and neck LELC is significantly better than nasopharyngeal LELC. Local treatments for LELC could be recommended according to primary tumor sites.

Primary pulmonary lymphoepithelioma-like carcinoma: a rare type of lung cancer with a favorable outcome in comparison to squamous carcinoma.

BACKGROUND: Primary pulmonary lymphoepithelioma-like carcinoma (LELC) is a rare tumor and often misdiagnosed as squamous carcinoma. In the current study, clinical characteristics and outcome of primary pulmonary LELC were systematically compared with pulmonary squamous carcinoma. METHODS: Forty-two cases of primary pulmonary LELC and 134 squamous carcinomas were enrolled retrospectively. Characteristic and prognosis difference between the two groups was compared, and the independent prognostic factor for pulmonary LELC was identified as well. RESULTS: In comparison to squamous carcinoma, pulmonary LELC was more common in women with a younger median age and less smokers. LELC seemed to be smaller in diameter on computed tomography (CT) scans than squamous carcinoma, with scarce spiculation and vascular convergence signs. Epstein-Bar virus-encoded RNA (EBER) by in-situ hybridization was detected in 33 LELC cases, among whom 27 ones were positive in serum EBV-DNA examination. LELC patients presented a much longer median progression-free survival (PFS) than squamous carcinoma. Positive serum EBV-DNA, distant lymph node invasion, advanced clinical stage and receiving radiotherapy were correlated with the shorter PFS in LELC patients. However, only positive serum EBV-DNA was the independent PFS predictor. CONCLUSION: Pulmonary LELC looks like distinct from squamous carcinoma. Middle-aged women and nonsmokers are comparatively predominated. CT features of pulmonary LELC are relatively less-malignant. Correspondently, the progression of pulmonary LELC is seemingly favorable than squamous carcinoma and the positive serum EBV-DNA appears to be the predictor of PFS.

A case of Lymphoepithelioma-like carcinoma (LELC) developed in the rectum.

Lymphoepithelioma-like carcinoma (LELC) is histologically similar in form to nasopharyngeal carcinoma (NPC) and is an epithelial tumor that is suggested to be involved in infection with Epstein-Barr virus (EBV), but it is rare to occur in the colon. A 35-year-old woman was found to have a rectal wall thickening by follow-up computed tomography (CT) image after sigmoidectomy and left salpingo-oophorectomy. Biopsy under colonoscopy revealed recurrence of ovarian cancer, and she underwent a low anterior resection. Pathological diagnosis was LELC. Although LELC of the stomach has been reported to have a high EBV infection rate as NPC, EBV infection was not detected in our case. Pelvic lymph node dissection was also performed, and metastasis was recognized around the iliac artery. There have been few reports of LELC occurring in the rectum, and there are no reports of distant metastasis only to the pelvic lymph node. We consider it a very valuable case, and report it with literature references.

Contrast-enhanced CT and PET-CT characteristics of primary tracheal lymphoepithelioma-like carcinoma: case series.

Background: Primary tracheal lymphoepithelioma-like carcinoma (LELC) is extremely rare, with only a few cases reported so far, and few studies have focused on the radiological features. This study aimed to investigate contrast-enhanced computed tomography (CECT) and positron emission tomography-computed tomography (PET-CT) presentations of primary tracheal LELC to improve diagnosis. Methods: A retrospective analysis was conducted on the clinical and imaging data of 13 patients with confirmed primary tracheal LELC between December 2013 and August 2022. We analyzed the radiological profiles of lesions on the CECT and PET-CT images. Results: In 92.3% (12/13) of the cases, primary tracheal LELC lesions predominantly occurred in the thoracic segment. They manifested as singular, wide-based, eccentric, irregular nodules, or exhibited mass-like thickening of the tracheal wall with invasive growth both internally and externally along the wall. The thickest dimension of the lesion ranged from 9 to 28 mm, affecting a length of 30.8±13.5 mm. Luminal stenosis was evident in all patients, with the narrowest point reaching a stenosis rate of 85%. Lesion margins were clear in 69.2% (9/13), indistinct in 23.1% (3/13), and unclear in 7.7% (1/13) of all cases. Among the patients, 92.3% (12/13) exhibited a relatively uniform density on CT plain scans, with a CT value of 44.5±7.8 Hounsfield units (HU). Enhancement scans revealed moderate to marked enhancement in 75% (9/12) of cases. In 2 cases undergoing PET-CT examination, lesion standardized uptake values (SUVs) were 4.4 and 5.1, whereas enlarged lymph node SUVs were 7.7 and 6.3, respectively. Mediastinal lymph node enlargement was observed in 8 patients (61.5%, 8/13), with a maximum short axis of 11.1±5.5 mm. After treatment, 9 out of 12 patients (75%) showed no evidence of distant metastasis upon CT re-examination. Conclusions: Early detection of primary tracheal LELC allows for curative resection and may lead to a favorable prognosis. It presents with characteristic CT findings, and the utilization of PET-CT improves diagnosis and staging.

Lymphoepithelial like carcinoma of the anorectal junction, a rare entity associated with human papillomavirus rather than Epstein-Barr virus: a case report.

Background: The anal canal is a rare site for lymphoepithelial-like carcinoma (LELC) and has only been reported in two patients. LELC is an undifferentiated malignant neoplasm with lymphoid background that has been reported at various sites like salivary glands, breast etc. however very few cases have been reported in gastrointestinal (GI) tract. Case Description: We report the third case of a 58-year-old female who presented with a complaint of mild constipation and rectal pressure for eight months. Endoscopic ultrasound (EUS) showed a 0.8 cm nodule at the anorectal junction which was resected. Pathology showed LELC of the anorectal junction with diffuse positivity with p16 immunostaining. Polymerase chain reaction (PCR) for human papillomavirus (HPV) was positive for high-risk HPV-16, in situ hybridization (ISH) for Epstein-Barr virus (EBV)-encoded ribonucleic acid (EBER) was negative, and there was no loss of nuclear expression of mismatch repair (MMR) proteins. Patient subsequently received chemoradiation with no evidence of residual disease on restaging scans. Conclusions: The association between LELC of anal canal and viral agents such as HPV or EBV has yet to be established; however, all three cases reported to date, including our current case, were positive for high-risk HPV-16, suggesting a possible role of HPV in tumorigenesis of LELC of the anal canal.

HPV-16-associated lymphoepithelial-like carcinoma mimicking rectal tonsil.

Lymphoepithelial-like carcinoma is a rare malignancy characterized by lack of cellular differentiation and associated nonneoplastic lymphoplasmacytic cell infiltrate that is rarely seen in the colon. Although many cases are associated with EBV infection, HPV may be present in LELC arising in sites known for HPV-driven malignancies, like the anogenital region. We report a case of lymphoepithelial-like carcinoma mimicking a rectal tonsil in a 51-year-old female. Attentive evaluation must be taken to identify this tumor in locations where prominent lymphoid stroma is an expected finding.

Novel Multimodal Management of Post-Partum Synchronous Metastatic Pulmonary EBV-Associated Lymphoepithelioma-Like Carcinoma (LELC)-A Case Report.

Primary Epstein-Barr-Virus (EBV)-associated pulmonary lymphoepithelioma-like carcinoma (LELC) is an aggressive rare cancer. Higher incidences have been observed in Asian sub-populations. Multimodal treatment paradigms have emerged as promising novel strategies in the management of advanced NSCLC. In this report, we describe the case of a 34-year-old female patient of Asian origin with a post-partum initial diagnosis of pulmonary LELC. Multimodal treatment with chemoimmunotherapy and hypofractionated irradiation to the primary tumour and main metastatic sites led to a favourable response demonstrating that radiotherapy may potentially augment anti-tumour immunity. To the best of our knowledge, this is the first case report on this novel therapy strategy of multi-site hypofractionated radiotherapy and chemoimmunotherapy for metastatic pulmonary EBV-associated LELC.

Primary pulmonary lymphoepithelioma-like carcinoma with positive expression of Epstein-Barr virus and PD-L1: A case report.

INTRODUCTION AND IMPORTANCE: Pulmonary lymphoepithelioma-like carcinoma (LELC) is a rare type of non-small cell lung cancer (NSCLC) that is classified as a subtype of unclassified carcinoma by the WHO. LELC is usually associated with Epstein-Barr virus (EBV) infection. LELC has often been observed in Southeast Asia; however, it is extremely rare in Japan. CASE PRESENTATION: A 60-year-old Japanese woman presented with an abnormal shadow in the left lung on chest radiography. Chest computed tomography showed a nodule located between the lingular and basal anteromedial segments. A blood test suggested an existing EBV infection, and LELC was suspected preoperatively in the transbronchial lung biopsy. She underwent a lingular and basal bi-segmentectomy. The EBV-encoded small ribonucleic acid in-situ hybridization (EBER-ISH) was positive, and she was diagnosed with LELC. Moreover, programmed death-ligand 1 (PD-L1) expression was moderately positive. No recurrence was observed for 30 months. CLINICAL DISCUSSION: Although LELC has been reported as a low-grade malignancy with a good prognosis, the frequency of PD-L1 expression in LELC seems to be higher than that in other NSCLCs. Moreover, it has been reported that LELC patients with high PD-L1 expression are likely to have early recurrence/metastasis and poor prognosis. CONCLUSION: An investigation of PD-L1 expression for LELC would be useful considering the benefit of PD-1/PD-L1 blockade in patients with pulmonary LELC with high PD-L1 expression. The present case is the first report of LELC with positive expression of EBER-ISH and PD-L1 in Japan.

Partial absence of PD-1 expression by tumor-infiltrating EBV-specific CD8+ T cells in EBV-driven lymphoepithelioma-like carcinoma.

OBJECTIVES: Lymphoepithelioma-like carcinoma (LELC) is an uncommon lung cancer, typically observed in young, non-smoking Asian populations. LELC is associated with Epstein-Barr virus (EBV) infection of lung tumor cells of epithelial origin, suggesting a carcinogenic role of EBV as observed in nasopharyngeal carcinoma (NPC). Here, we studied the antigen specificity and phenotype of EBV-specific CD8+ T cells in blood and tumor of one LELC patient positive for EBV infection in lung tumor cells. METHODS: Using multiplex MHC class I tetramers, mass cytometry and mRNA sequencing, we studied EBV-specific CD8+ T cells at the transcriptomic and phenotypic levels in blood and tumor tissues of the LELC patient. RESULTS: Lymphoepithelioma-like carcinoma lung tumor cells were positive for EBV infection. In both blood and tumor tissues, we detected two populations of EBV-specific CD8+ T cells targeting the EBV lytic cycle proteins: BRLF1 and BMLF1. Transcriptomic analyses of these two populations in the tumor, which can be considered as tumor-specific, revealed their distinct exhausted profile and polyclonal TCR repertoire. High-dimensional phenotypical analysis revealed the distinct phenotype of these cells between blood and tumor tissues. In tumor tissue, EBV-specific CD8+ TILs were phenotypically heterogeneous, but consistently expressed CD39. Unexpectedly, although the LELC tumor cells expressed abundant PD-L1, these tumor-specific CD8+ tumor-infiltrating lymphocytes (TILs) mostly did not express PD-1. CONCLUSION: Epstein-Barr virus-specific CD8+ TILs in EBV-driven tumor are heterogeneous and partially lack PD-1 expression, suggesting that anti-PD1/PD-L1 immunotherapy may not be an appropriate strategy for disinhibiting EBV-specific cells in the treatment of LELC patients.

Primary Pulmonary Lymphoepithelioma-Like Carcinoma Response Favorably To Nivolumab: A Case Report.

OBJECTIVES: Lymphoepithelioma-like carcinoma (LELC) is a rare subtype of non-small cell lung cancer (NSCLC). No clinical trials have been performed, and no course of treatment for LELC has been established because of it's rarity. This study presents a patient with primary pulmonary LELC, who was treated with nivolumab, and responded favorably. MATERIALS AND METHODS: A patient with primary pulmonary LELC was treated using nivolumab. RESULT: The patient responded well to immunotherapy with nivolumab. After five cycles of the nivolumab, the size of the tumor and the lesions of the liver became smaller. A blood test showed that CYFRA21-1 and NSE had dramatically decreased from before, especially the CYFRA21-1. CONCLUSION: EBV-positive pulmonary LELC with high expression of PD-L1 may derive a benefit from PD-1/PD-L1 blockade.

Clinicopathological characteristics with EGFR, ALK, ROS1 genetic alternation and prognostic analysis of primary lymphoepithelioma-like carcinoma.

BACKGROUND: Primary lymphoepithelioma-like carcinoma (LELC) is a rare form of lung cancer, since genetic alternations participate in the carcinogenesis in lung cancer, whether critical driver genes such as ROS1 fusion, anaplastic lymphoma kinase (ALK) rearrangement and epidermal growth factor receptor (EGFR) mutation play roles in LELC remains unclear. METHODS: We collected a total of 30 LELC samples for genetic and prognosis analysis retrospectively in Shanghai Chest Hospital, EGFR gene mutation, ALK rearrangement and ROS1 fusion status were extracted from digital database. Clinicopathological characteristics with genetic profiling and survival were analyzed. RESULTS: All samples appeared negative for genes (EGFR, ALK and ROS1) alternations detection. Female gender acted as an independently prognostic factor in poorer disease-free survival (DFS) (P=0.034), and tumors locate in the left lobe associate with worse DFS in univariate analysis (significant trend, P=0.051), moreover, serum positive NSE level also indicate a shorter DFS after adjustment (significant trend, P=0.057). Most of LELC are diagnosed at early stage, with 90.0% (27/30) patients obtained the opportunities for surgery. CONCLUSIONS: Since no classical genetic alternations appeared in present study, more investigations of other genes distributions should be explored in the future for better understanding of this rare subtype of lung cancer. Serum positive neuron-specific enolase (NSE) level seemed to be a prognostic biomarker for DFS in LELC patients.

Correlation and prognostic significance of PD-L1 and P53 expression in resected primary pulmonary lymphoepithelioma-like carcinoma.

BACKGROUND: Aberrant expression of programmed cell death-ligand 1 (PD-L1) and protein 53 (P53) has been observed in various malignancies, and recently, the mechanism of PD-L1 regulation by P53 has been elucidated. We aimed to explore possible correlations between PD-L1 and P53 expression and the prognosis of patients with resected pulmonary lymphoepithelioma-like carcinoma (LELC). METHODS: A total of 67 consecutive patients with primary pulmonary LELC who underwent radical resection from January 2003 to December 2014 were enrolled in our study. Membranous PD-L1 and nuclear P53 expression were detected by immunohistochemical staining (IHC). RESULTS: Positive expression of PD-L1 in tumor cells (TCs), PD-L1 in tumor-infiltrating lymphocytes (TILs) and P53 was investigated in 44 patients (65.7%), 37 patients (55.2%), and 34 patients (50.7%), respectively. Using univariate and multivariable analysis, both PD-L1 (+) in TCs and P53 (+) were observed to be significantly independent prognostic factors associated with longer disease-free survival (DFS, P=0.037 and 0.039, respectively), along with early stage LELC (P=0.037), but had no association with overall survival (OS) (P>0.05). In the P53 (+) group, the rate of patients with PD-L1 (+) in TCs was significantly higher than in the P53 (-) group (85.3% vs. 45.5%, P=0.001). In addition, among the 45 patients who underwent adjuvant chemotherapy, DFS was significantly longer in patients with either PD-L1 (+) in TCs or P53 (+) (P=0.036 and 0.044, respectively). CONCLUSIONS: PD-L1 and P53 may be potential therapeutic targets for primary pulmonary LELC. PD-L1 (+) in TCs and P53 (+) were reliable predictors for longer DFS and benefits from adjuvant therapy in resected cases. Routine detection of these two indices in lung LELC may be warranted.

Response of advanced stage recurrent lymphoepithelioma-like carcinoma to nivolumab.

Lymphoepithelioma-like carcinoma (LELC) of lung is a rare tumor that is mostly reported in south-east Asian countries. The surgical removal is curative in the early stages but there is no consensus on the choice of chemotherapy for the treatment of advanced stage tumors. Most of the data on chemotherapy are based on small case series and retrospective studies. As per available data, this tumor responds to chemotherapy initially but recurrences are common. The use of conventional chemotherapy in recurrent tumors leads to cumulative toxicities in the long term. Due to lack of actionable mutations, targeted therapies are also not very useful. Immune check point inhibitors immune checkpoint inhibitors have shown survival benefit in patients with advanced stage non-small-cell and small cell carcinoma with better side effect profile than conventional chemotherapy. The role of immune checkpoint inhibitors in LELC is unknown. Though several studies have reported high expression of programmed cell death-1 (PD-1)/or its -ligand (PD-L1) in LELC providing a rationale for trial of these agents, the actual benefit of these agents in LELC has not been reported so far. In this case series, we report two cases of advanced stage LELC that progressed despite multiple lines of chemotherapy but responded favorably to a PD-1 inhibitor, nivolumab.

Lymphoepithelioma-Like Carcinoma of the Breast Mimicking Granulomatous Mastitis- Case Report and Review of the Literature

Lymphoepithelioma-like carcinoma (LELC) of the breast is an exceedingly rare variant of mammary cancer. To our knowledge, only twenty - one cases have been reported in the literature. Diagnosis of this type of mammary carcinoma may be challenging, owing to its rarity and the histopathological similarity to common inflammatory and malignant lesions of the breast mainly granulomatous mastitis, medullary carcinoma, pleomorphic lobular carcinoma, lymphoma and other hematological malignancies. Our case is the 22nd case of lymphoepithelioma-like carcinoma reported in the breast, presenting with a palpable tender mass in a post-menopausal female. Her clinical picture had been mistaken for inflammatory disease. We present our case, with its detailed clinical history, radiological findings, histopathological and immune-histochemical findings along with a review of the literature. Highlighting this type of tumors may help in appropriate diagnosis. Moreover, studying the behavior of these rare neoplasms is essential to expedite treatment for this tumor type.

Distribution of lymph node metastasis from lymphoepithelial-like carcinoma of the parotid.

PURPOSE: To explore the distribution of node spread from lymphoepithelial-like carcinoma (LELC) of the parotid gland based on the 2013 updated guidelines for neck node levels. RESULTS: 42 (58.3%) cases had metastatic nodes, all were localized at the ipsilateral neck. The detailed distribution was: level Ia 0, level Ib 6(14.3%), level II 34 (80.1%), level III 16 (38.1%), level IV 9 (21.4%), level V 7 (16.7%), level VI 0, level VII 0, level VIII 37 (88.1%), level IX 0, level Xa 2 (4.8%), and level Xb 0. Lymphadenopathy in level Ib, V and Xa was always accompanied with level II or level VIII nodal metastasis. No statistical significance was found in the incidence of nodal involvement between T1-2 and T3-4 tumors (57.4% versus 61.1%, p = 0.78). METHODS: We retrospectively reviewed the surgical and imaging documents of 72 cases of LELC from the parotid gland between January 2004 and November 2015. All patients received contrast enhanced computed tomography (CT) or magnetic resonance imaging (MRI). Parotid metastasis from nasopharyngeal cancer (NPC) was excluded. Nodal status and distribution was evaluated by both pathologic reports and imaging studies. CONCLUSIONS: This is the first description of topography of cervical nodal metastases from LELC of the parotid gland. Incidence of nodal involvement is high. Nodes at ipsilateral level VIII and II were most frequently involved, followed by level III, IV, V and Ib. Nodes in level Ia, VI and level VII were rarely seen.

Advanced primary pulmonary lymphoepithelioma-like carcinoma: clinical manifestations, treatment, and outcome.

BACKGROUND: Primary pulmonary lymphoepithelioma-like carcinoma (LELC) is rare, with better clinical outcomes than other lung cancers. However, reports on advanced LELC characteristics and prognosis are lacking. METHODS: This retrospective study included adults diagnosed with advanced LELC (at least stage IIIA) between January 2003 and December 2015. Clinical characteristics, treatment modalities, and outcomes were recorded. RESULTS: Study population comprised 23 patients with a mean age of 63.7±10.6 years. The Eastern Cooperative Oncology Group status on diagnosis was 0 in five patients and 1 in the others. Most patients received multimodality treatment and all received cisplatin-based chemotherapy. Median follow-up duration was 28.8 months. The median progression free survival (PFS) was 14.6 months in patients received palliative chemotherapy. There were nine (39.1%) deaths. The median overall survival (OS) was not achieved. Until July 31, 2016, median OS was 54.1 months for stage IIIB and 27.6 months for stage IV. There was no significant difference in OS among all stages. No prognostic factors were found. CONCLUSIONS: Advanced LELC responded well to cisplatin-based chemotherapy and/or radiotherapy. Main tumor resection is probably beneficial for advanced LELC. Long-term survival is possible for advanced LELC after multimodality treatment.

Clinicopathological features and prognosis of primary pulmonary lymphoepithelioma-like carcinoma.

BACKGROUND: Lymphoepithelioma-like carcinoma (LELC) is a rare form of non-small cell lung carcinoma. The current study focused on its clinicopathological features and potential factors influencing the prognosis. METHODS: The statistical analysis was based on the clinicopathological records and the prognosis of 43 LELC patients, analyzed by Kaplan-Meier method, Log-rank test, and COX regression analysis. RESULTS: The patients' average age was 57.35±9.22 years, 86.05% of them were non-smokers and 53.49% were women. The average tumor diameter was 3.24±1.57 cm. The 2- and 5-year overall survival (OS) rates of LELC patients were 90% and 74%, respectively; the disease-free survival (DFS) rates were 87% and 47%, respectively. The patients with large tumor, accompanied with lymph nodes metastasis or at the advanced stage had the worst OS, and the patients with lymph nodes metastasis or at the advanced stage had the worst DFS. Univariate analysis indicated that T and N grading and TNM stage influenced the OS, and N grading and TNM stage influenced the DFS; the independent factors affecting OS or DFS were not identified by multivariate analysis. CONCLUSIONS: LELC commonly occurred in senior non-smoking women. In summary, the prognosis of LELC was satisfactory.

Metastatic lymphoepithelioma-like carcinoma of the lung treated with nivolumab: a case report and focused review of literature.

In recent years, significant advances have been made in cancer immunotherapy. Here, we present the first report of a patient with lymphoepithelioma-like carcinoma (LELC) of the lung, an Epstein-Barr virus (EBV)-associated lung cancer, who was treated with nivolumab, a fully human IgG4 anti-PD-1 monoclonal antibody. We also carry out a focused review to identify and examine studies of LELC of the lung in the literature. This case report highlights the need to further assess the role of immune checkpoint inhibitors in LELC of the lung.