Lymphoepithelioma-like Carcinoma Research Articles

Experience of long-term clinical observation of a patient with a rare type of stomach cancer – lymphoepithelioma-like carcinoma

Stomach cancer is one of the most frequently diagnosed cancers. The success of special methods of treatment are still disappointing in this disease, and stomach cancer has historically been considered a tumor refractory to treatment. Therefore, this category of patients, especially in the stage of dissemination, in most cases, palliative therapy is recommended. In recent years, the use of systemic chemotherapy and targeted therapy has led to a significant improvement in the quality of life and survival rates for stomach cancer compared to palliative therapy. However, significant progress in diagnostic methods over the past two decades has made it possible and possible to review approaches to the treatment of many cancer diseases that are refractory to chemotherapy. The last 2 decades, due to improved methods of diagnosis have allowed to revise approaches to the treatment of many refractory to chemotherapy of neoplastic diseases. In particular, the literature describes cases of revision and changes due to pre-existing diagnosis of IHC Research (carcinoid, NEO, stomach cancer, pancreatic cancer). This communication allows to share their own long experience of observation and treatment of a patient with a rare form of stomach cancer – lymphoepithelioma-like carcinoma.

Distinct Molecular Landscape of Epstein-Barr Virus Associated Pulmonary Lymphoepithelioma-Like Carcinoma Revealed by Genomic Sequencing.

Pulmonary lymphoepithelioma-like carcinoma (LELC) is a subtype of non-small cell lung cancer (NSCLC) characterized by marked lymphocytic infiltration and association with Epstein–Barr virus (EBV). The molecular basis underlying the disease remains unclear. We sought to study the molecular landscape by multiple approaches including whole genomic sequencing, capture-based targeted sequencing, fluorescent in situ hybridization and immunohistochemistry. Tumor cells from 57 EBV-positive pulmonary LELCs were isolated by careful microdissection prior to genomic sequencing. Integrated analysis revealed a distinct genomic landscape of low TP53 mutation rate (11%), low incidence of known drivers in the RTK/RAS/RAF (11%) and PI3K/AKT/mTOR pathways (7%), but enriched for loss-of-function mutations in multiple negative regulators of the NF-κB pathway. High level programmed cell death ligand-1 (PD-L1) expression was shown with 47% and 79% of the cases showing positive PD-L1 immunoreactivity at ≥50% and ≥1% tumor proportion score, respectively. Subsets of the patients with actionable fibroblast growth factor receptor 3 (FGFR3) aberrations (4%) and mismatch repair deficiency (4%) were potentially eligible for precision medicine. Pulmonary LELC showed a distinct genomic landscape, different from major NSCLC subtypes but resembled that of EBV-associated nasopharyngeal carcinoma. Our work facilitated the understanding of molecular basis underlying pulmonary LELC to explore potential therapeutic options.

The Clinicopathological Features and Genetic Alterations in Epstein-Barr Virus-Associated Gastric Cancer Patients after Curative Surgery.

Background: Epstein–Barr virus (EBV)-associated gastric cancer (GC) is one of four major gastric cancer types and is traditionally considered to be related to lymphoepithelioma-like GC. Few studies have investigated the clinical significance of EBV infection in intestinal/solid type, diffuse (poorly cohesive) type, and lymphoepithelioma-like GC. Methods: A total of 460 GC patients receiving curative surgery were enrolled. The clinicopathological features, genetic alterations and prognoses were compared between patients with and without EBV infection. Results: EBV-positive GC patients (n = 43) had more tumors located in the upper and middle stomach, more common in lymphoepithelioma-like carcinoma, more lymphoid stroma, fewer Helicobacter pylori infections, and higher programmed death-ligand 1 (PD-L1) expression than EBV-negative GC patients. For intestinal/solid type GC, EBV-positive tumors were more likely to be located in the upper and middle stomach, have more lymphoid stroma, fewer Helicobacter pylori infections, higher PD-L1 expression, and more liver metastases than EBV-negative tumors. For diffuse (poorly cohesive) type GC, EBV-positive tumors were more likely to be located in the upper stomach, and have more lymphoid stroma than EBV-negative tumors. For lymphoepithelioma-like GC, EBV-positive tumors had more PI3K/AKT pathway mutations than EBV-negative tumors. Conclusions: Intestinal/solid type GC patients with EBV-positive tumors were associated with higher PD-L1 expression and more liver metastases, while lymphoepithelioma-like GC patients with EBV-positive tumors had more PI3K/AKT pathway mutations. Immunotherapy and targeted therapy may be beneficial for these groups of patients. Routine EBV survey is recommended in GC.

Primary pulmonary lymphoepithelioma-like carcinoma is characterized by high PD-L1 expression, but low tumor mutation burden

Primary pulmonary lymphoepithelioma-like carcinoma (PLELC) is a rare subtype of non-small cell lung cancer (NSCLC). There are few reported studies on the relationship between programmed death ligand-1 (PD-L1) expression and genomics features of this distinct NSCLC subtype. Our study aimed to investigate the expression levels of PD-L1 to determine their clinical value and to identify genetic alterations in PLELC. Fifty-nine PLELC patients, whose clinical information and pathology results were available, were included in this study. Immunohistochemical analysis of PD-L1 was performed in all cases. Specimens of 37 PLELCs and 3 metastatic nasopharyngeal carcinomas (NPCs) of the lung, resected within the previous 3 years, were chosen for mutation analysis, using next-generation sequencing of 425 genes. PLELC patients in the present study were mainly non-smoking females, with a high frequency of PD-L1 positivity in their tumors. Positivity rates were 96.6 %, 91.5 %, 83.1 %, and 61.0 % at tumor proportion scores (TPSs)≥ 1%, 5%, 10 %, and 50 %, respectively. Moreover, we observed that PD-L1 expression was higher in specimens stored for ≤ 3 years and in tumor cells with vesicular nucleus morphology at a TPS ≥ 50 %. Mutation analysis suggested a relatively high frequency of TP53 mutations and MCL1 copy number variation, but low tumor mutation burden (TMB) (ranging from 0 to 6.9, median of 1.1 mutation per megabase) and similarity of gene alteration with NPCs. However, no specific germline mutation was detected in PLELC patients. Additionally, survival analysis showed that patients in the early stages (stage I and II) had higher progression-free survival rates (P = 0.035) and those with tumors containing obvious stroma fibrosis tended to have worse prognosis (P = 0.008). However only stage was shown to be the independent prognostic factor (P = 0.008, HR=4.807, 95 %CI:1.508−15.323).PLELC is a subtype of lung cancer with distinct clinicopathological and genetic features, especially characterized by high PD-L1 expression and low TMB.

Hepatic lymphoepithelioma-like carcinoma: a case report with literature review

: Primary hepatic lymphoepithelioma-like carcinoma (LELC) is rare. Here we report a case of a 39-year-old woman who presented with abdominal pain and weight loss for one month. A space-occupying lesion was demonstrated in the left liver lobe by MRI. Further laboratory studies showed abnormal liver function tests. A partial hepatectomy including the lesion was performed, with clear margin. A well demarcated gray-white nodule was found, measuring 15 mm × 12 mm × 10 mm, with no satellite nodules. Microscopically, the tumor consisted of neoplastic cells arranged in nest and sheets, surrounded and infiltrated by abundant lymphoid tissue. Immunohistochemical analyses showed the tumor to be positive for CEA, CK7, CK19, and villin. The Ki-67 index was about 30%. In situ hybridization for EBER was positive. A comprehensive examination including EGD with extensive biopsies of the stomach did not reveal another lesion. Therefore, this represented a primary Epstein-Barr virus (EBV)-associated hepatic LELC. No recurrence was found followed up one year. Liver is a common target for metastasis, when a liver tumor with abundant lymphocytes infiltrated is found in adults with EBV positive, the possibility of metastatic carcinoma must be excluded before the tumor can be categorized as primary, and the possibility of EBV-related LELC should be considered.

Computed tomography-based differentiation of primary pulmonary lymphoepithelioma-like carcinoma and small-cell lung cancer.

Background:Primary pulmonary lymphoepithelioma-like carcinoma (LELC) is a rare subtype of lung cancer. Both small-cell lung cancer (SCLC) and LELC often manifest as a centrally located tumor with lymphadenopathy. This retrospective study investigated and compared the initial computed tomography (CT) features and subsequent survival outcomes of LELC and SCLC.Methods:A total of 50 patients with a confirmed diagnosis of LELC were enrolled and matched at a ratio of 1:1 with patients with SCLC according to the tumor stage. Utilizing a consensus approach, two radiologists reviewed pretreatment CT images. Survival outcomes were analyzed.Results:Well-defined tumors were significantly more common in the LELC group (LELC: 42% vs SCLC: 24%, p = 0.005). Based on the comparisons of the primary tumor with the muscles, LELC tumors exhibited a significantly higher percentage of attenuation on contrast-enhanced CT scans (21.6% ± 29% vs −14.2% ± 37%, p < 0.001). The prevalence of vascular or bronchial encasement (18% vs 40%, p = 0.028), background emphysematous changes (10% vs 60%, p < 0.001), and tumors located in upper lobes (18% vs 64%, p < 0.001) was significantly lower in the LELC group. Female gender (70% vs 12%, p < 0.001), younger age (57.6 ± 12.0 years vs 68.0 ± 11.0 years, p < 0.001), and without a history of smoking (16% vs 88%, p < 0.001) were factors more commonly found in the LELC group. The patients with LELC had a better prognosis with significantly longer median survival than did the patients with SCLC (23.4 months vs 17.3 months, p = 0.01).Conclusion:Because SCLC demonstrated a more aggressive disease progression, differentiating LELC from SCLC is crucial. In Epstein–Barr virus-endemic areas, the diagnosis of LELC should be considered when approaching a patient with the above-mentioned CT and clinical features.

Rare primary lymphoepithelioma-like carcinoma of the renal pelvis.

Lymphoepithelioma-like carcinoma (LELC) is a rare, malignant epithelial tumour which can arise within the upper urinary tract. This letter adds to a previous systematic review and cumulative analysis of 28 published upper urinary tract-LELC cases which provided insight into this disease; however, the current evidence does not provide clinicians with clear guidelines due to its rarity. Therefore, the aim was to report a new case of renal pelvis LELC presented in our hospital. In this instance, we were able to report treatment experience and long-term follow-up results. This patient presented with hypertension and haemturia which initiated further investigation. While ultrasound identified an hypechoic mass, no malignant cells were detected using cytological testing. Abdominal magnetic resonance imaging identified a slightly enhanced mass in the left renal pelvis with no evidence of lymph node metastasis. Ureteroscopic tumor biopsy suggested the existence of urothelial carcinoma, hence, laparoscopic radical left nephroureterectomy with bladder cuff excision was performed. Through patient-practitioner consultations, we decided to adopt a "watch and wait" approach after radical nephroureterectomy rather than administering chemotherapy. Although, we would encourage clinicians to record and publish cases to garner insight into this type of malignant disease.

&lt;p&gt;Pulmonary Lymphoepithelioma-Like Carcinoma: A Mini-Review&lt;/p&gt;

Pulmonary lymphoepithelioma-like carcinoma (PLELC) is a rare and distinct subtype of non-small-cell lung carcinoma associated with Epstein–Barr virus (EBV) infection. We systematically reviewed the recent research that expands our knowledge about PLELC, with main focus on its genetic profile, tumor-infiltrating environment, PD-L1 expression, circulating EBV-DNA, clinical utility of 18F-FDG PET/CT, and treatment strategy. A low frequency of typical driver mutations and widespread existence of copy number variations was detected in PLELC. Persistent EBV infection may trigger intense infiltration of lymphocytes, representing enhanced tumor immunity and possibly resulting in a better prognosis. Circulating EBV-DNA in the plasma of patients with PLELC may predict disease progression and response to therapy. PLELC is 18F-FDG avid, and 18F-FDG PET may help refine palliation strategies and subsequently improve the prognosis. Most of the reported patients present at early and resectable stage, and surgical resection with curative intent is the preferred approach. There is currently no consensus on the regimen of chemotherapy for patients with advanced stages. EGFR-targeted therapies seem to have no therapeutic effect, and the clinical impact of PD-1/PD-L1 therapy is uncertain but worthy of further research.

Clinical Analysis of Primary Pulmonary Lymphoepithelioma-like Carcinoma in 8 Patients

背景与目的淋巴上皮瘤样癌为罕见的上皮肿瘤，多来源于鼻咽部，也发生在前肠起源器官，如肺、胃、唾液腺和胸腺。原发性肺淋巴上皮瘤样癌（primary pulmonary lymphoepithelioma-like carcinoma, PPLELC）是一种罕见的非小细胞肺癌（non-small cell lung cancer, NSCLC），约占肺癌的0.9%。本文目的在于探讨PPLELC的临床病理特点、治疗方法以及预后情况。方法回顾性分析湖南省肿瘤医院2013年10月-2016年6月收治的PPLELC患者8例，对其临床资料及预后进行临床分析，以深入研究这种罕见的肺癌亚型。结果8例患者中，62.5%（5/8）为女性，中位年龄为51.5岁（41岁-64岁），大多数（87.5%）患者不吸烟，50.0%患者无症状，约37.5%为Ⅰ期，50.0%为Ⅲ期，12.5%为Ⅳ期。PPLELC的典型病理特征与未分化的鼻咽癌相同，其特点是分化较差的肿瘤细胞，具有大的泡状核和核仁突出，呈合胞体生长，伴有淋巴细胞浸润。免疫表型：癌细胞P63（100.0%, 6/6）、CK5/6（100.0%, 5/5）、CK（100.0%, 5/5）阳性。对8例患者的程序性死亡配体-1（programmed cell death-ligand 1, PD-L1）表达进行了检测，当≥5%的细胞膜染色时定义为PD-L1阳性，PD-L1表达频率为50.0%（4/8），肿瘤细胞阳性比例分数（tumor proportion score, TPS）波动在20.0%-70.0%。3例行表皮生长因子受体（epidermal growth factor receptor, EGFR）基因突变检测，结果呈阴性；2例行EML4-ALK融合基因检测，结果呈阴性；1例行KRAS、B-raf、C-kit、HER2、VEGFR1、VEGFR2检测，结果示KRAS野生型，B-raf、C-kit无突变，HER2、VEGFR1、VEGFR2中等表达。所有患者均接受了手术治疗，其中接受了辅助化疗者5例，其中Ib期2例，Ⅲa期2例，Ⅳ期1例；辅助放化疗者1例，分期为Ⅲa期，接受了新辅助化疗者1例，分期为Ⅲa期。截止至随访截止期，所有患者均存活，中位存活时间为57个月，3年和5年总生存率（overall survival, OS）分别为87.5%和50.0%；无病生存率（disease-free survival, DFS）分别为87.5%和50.0%。结论PPLELC是一种罕见但独特的NSCLC亚型，具有明显的临床病理学特征，通常发生于年轻的非吸烟患者，无性别倾向，并与EB病毒（Epstein-Barr virus, EBV）感染有关，组织形态及免疫组化是诊断的主要手段。大多数患者缺乏EGFR和ALK等常见基因突变，提示这些基因的突变与PPLELC的发生无关。PD-1和PD-L1可能是潜在的治疗靶点。与其他类型的NSCLC相比，大多数患者处于早期或局部晚期，预后较好。目前还没有针对这种罕见肿瘤的标准化治疗方案。早期以手术切除为主，中晚期或转移阶段则采用手术、化疗、放疗等多种治疗方式。由于其发病率低，为了确定其生物学特性和最佳治疗方案，还需要进一步的研究。

Primary pulmonary lymphoepithelioma-like carcinoma.

Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is an extremely rare subtype of non-small-cell lung cancer (NSCLC) but differs from other NSCLC types. Most patients with PPLELC have almost no obvious clinical manifestations, and are often diagnosed in the advanced stage. Complete resection is the standard treatment for cases with early stage disease, while chemotherapy and radiotherapy are effective to some extent in late stage disease. High expression of PD-L1 in PPLELC tumor cells confers immunotherapy an important role in the treatment of PPLELC, and programmed death-1 (PD-1) inhibitors such as nivolumab and pembrolizumab may be optimal options for PPLELC patients. This review focuses on the clinical and pathologic features, treatment, and prognosis of PPLELC.

Lymphoepithelioma-like carcinoma of the upper urinary tract: A systematic review of case reports.

BACKGROUNDLymphoepithelioma-like carcinomas (LELCs) are rare, malignant epithelial tumors, generally considered a subtype of squamous cell carcinoma. LELCs are undifferentiated and can occur in multiple tissues, although LELCs in the urinary tract are extremely rare. As such, evidence does not provide clinicians with guidelines for the best practices. Even though this is a rare disease, it is associated with high morbidity and mortality. Therefore, we must learn to differentiate LELC types and identify risk factors for early identification.AIMTo develop an evidence base to guide clinicians treating primary LELCs of the upper urinary tract (UUT-LELC).METHODSWe performed a systematic review of all reports on UUT-LELC from the first published case in 1998 until October 2019, according to the PRISMA. A database was then developed by extracting data from previously published reports in order to analyze interactions between clinical characteristics, pathological features, interventions and outcomes. Survival was analyzed using Kaplan–Meier estimates, which were compared using log rank tests.RESULTSA total of 28 previously published cases were identified for inclusion. The median age was 72 years with a male to female ratio of 4:3. Pure type LELCs were most common with 48.3% (n = 14), followed by 37.9% (n = 11) predominant LELCs and 3.4% (n = 1) focal LELCs. Epstein-Barr virus testing was negative in all cases. Fourteen patients received radical nephroureterectomy (RNU)-based intervention. Twenty-three patients survived with no evidence of further metastasis, although six died before the median 18 mo follow-up point. Survival analysis suggests pure histological subtypes, and patients who receive complete tumor resection have more favorable prognoses. As always in cancer care, early identification generally increases the probability of interventional success.CONCLUSIONThe most effective treatment for UUT-LELC is RNU-based therapy. Since cases are few in number, case reporting must be enhanced and publishing encouraged to both save and prolong lives.

Clinicopathological features of multifocal gastric carcinoma: A retrospective study.

453 Background: The occurrence of multifocal gastric carcinoma (MGC) is growing in China, while relevant study remains limited. This study aimed to collect more information of MGC for further research. Methods: Patients with MGC treated at China National Cancer Center from January 2010 to December 2017 were enrolled in this retrospective study. A 6-month interval was used to separate synchronous and metachronous foci. Results: 103 patients were included. 88 (85.4%) were males. 96 (93.2%) patients had two foci and 7 (6.8%) had three or more foci, contributing a total of 216 tumor foci. 185 (85.6%) foci were adenocarcinoma, 18 (8.3%) were intraepithelial neoplasia, 2 (0.9%) were lymphoepithelioma-like carcinoma and 1 (0.5%) was small cell carcinoma. Intestinal, diffuse, and mixed type accounted for 49.7%, 14.6% and 11.4% respectively, with 24.3% unknown.In 96 patients with 2 foci, 56 (58.3%) patients had synchronous diseases, and 40 (41.7%) had metachronous diseases. The median age at the diagnosis of first tumor was 62 (55-71) years. The median diagnosis interval of metachronous tumor foci was 41.4 (23.5-88.3) months. The locations and sizes of foci are shown in Table. In synchronous cases, 6 foci of accessory tumor were less than 1cm including one being 0.4 cm. In metachronous cases, 4 foci of second tumor were less than 1 cm. 51 (49.5%) patients were current smokers or ex-smokers, and 44 (42.7%) were regular alcohol consumers. 26 (25.2%) patients had a first-relative family history, including 14 (13.6%) having a family history of gastrointestinal carcinoma. Conclusions: The second gastric tumor should be thoroughly detected to avoid missed diagnosis, since the accessory tumor might be rather small. Further genetic research is warranted to explore the potential pathogenesis of MGC. [Table: see text]

The clinical analysis of pulmonary lymphoepithelioma-like carcinoma with epithelioid granuloma.

BackgroundPulmonary lymphoepithelioma-like carcinoma (PLELC) is a rare subtype of non-small cell lung cancer associated with Epstein-bar virus (EBV) infection. Epithelioid granuloma (EG) has been more scarcely reported and frequently misdiagnosed.MethodsData were collected from January 2013 to October 2019. Of 227 patients diagnosed as having PLELC, 22 patients had EG. We analyzed their clinical features, pathological characteristics and treatment and a comparison between PLELC patients complicated with or without EG was made.ResultsTwenty-two patients had complicated with EG (9.6%). The median age was 50 years (38–67 years). There were more females than males (1.4:1). Most patients were at early stage (68.2%) with nonspecific manifestations and lack of Rich-Lewis phenomenon. Compared with the 205 patients complicated without EG, there were no significant difference among age (t=0.938, P=0.349), gender (χ2=0.898, P=0.343), initial symptoms (χ2=2.684, P=0.443), smoking status (χ2=0.210, P=0.647), diameter of tumor(t=0.993, P=0.332) and performance status (H=0.971, P=0.615). EG was often located inside or adjacent to the tumor (71.4%). Specific staining was negative, whereas in situ hybridization staining of EBV-encoded RNA was consistently positive. Most patients complicated with EG received multimodality therapy including surgery, neo-adjuvant/adjuvant chemotherapy or palliative chemotherapy and none of them received anti-TB therapy. Compared with the 205 patients complicated without EG, there were no significant difference among tumor stage, DFS (median, not reached, P=0.914), PFS (median, 12.3 months, P=0.848), OS (median, not reached, P=0.737) and treatment including anti-tumor therapy and anti-TB therapy. During follow-up duration for 14.6 months (range, 2.1–94.7 months), none of the patients had occurrence, progression or relapse of tuberculosis, regardless whether anti-tuberculosis therapy was initiated.ConclusionsPLELC complicated with EG was lack of Rich-Lewis phenomenon and specific clinical characteristics compared with those without EG. EG might be caused by immunological hypersensitivity to tumor cells or EBV infection but not pulmonary tuberculosis. PLELC complicated with EG could be treated with chemotherapy and surgery. However, anti-tuberculosis therapy was unnecessary.

Lymphoepithelioma-like Gastric Carcinoma a Rare Condition: A case report

Background: Lymphoepithelioma-like gastric carcinoma (LELGC) is a rare type of gastric cancer characterized by a carcinoma with intense stromal lymphocytic infiltration. It comprises approximately 4% of all gastric neoplasms and occurs mainly in men and in the proximal stomach. LELGCs have a better prognosis than other gastric carcinomas due to its lower invasive and metastatic capacity. Unfortunately, there is a lack of diagnostic criteria that help identifying this tumor and differentiating it from medular and tubular adenocarcinoma with lymphoid stroma. Epstein-Barr Virus (EBV) and microsatellite instability have a predominant role in the physiopathology of the disease. The clinical manifestations are similar to those of other gastric neoplasms.

Special histopathological variants and potential diagnostic traps of classical follicular dendritic cell sarcoma

Objective: To investigate the clinicopathological features, special morphologic variants and potential diagnostic traps of classical follicular dendritic cell sarcoma (FDCS). Methods: A total of 25 cases of classical FDCS diagnosed in the First Hospital Affiliated to Army Medical University from 2006 to 2018 were examined by hematoxylin-eosin staining, immunohistochemistry and in situ hybridization for Epstein-Barr virus-encoded mRNA (EBER). Meanwhile, the types and characteristics of the special variants of FDCS were summarized along with those reported in the literature. Results: The age of patients ranged from 23 to 77 years (mean 52 years), the male to female ratio was 1.5, and the maximum diameter of tumor was 1.5 to 20 cm (mean 7.4 cm). Twelve cases (48%) were misdiagnosed at the initial evaluation. Follow-up information was available for 17 patients, and the follow-up time was 5 to 96 months. The propotion of patients having recurrence, metastasis and mortality was 3/17, 5/17 and 2/17, respectively. Microscopically, besides the typical morphology, 10 cases of FDCS showed special histomorphologies and/or structures, including those mimicking lymphoepithelioma-like carcinoma, desmoplastic infiltrating carcinoma, classical Hodgkin's lymphoma (CHL), anaplastic large cell lymphoma (ALCL) and hemangiopericytoma. These morphologic variants were potential diagnostic pitfalls and warranted attention. Immunohistochemistry showed that more than two markers of follicular dendritic cells (such as CD21, CD23, CD35, etc.) were expressed in cases showing typical morphology and the special variants. All 25 cases were all negative for EBER by in situ hybridization. Conclusions: Classical FDCS is rare, besides the typical morphologic features, there are many special variants. In particular, these may be confused with lymphoepithelioma-like carcinoma in the nasopharynx, CHL or ALCL in the mediastinum/lymph node. Awareness of these variants is essential for accurate diagnosis.

Clinicopathological features of the lymphoepithelioma-like carcinoma

目的： 探讨淋巴上皮瘤样癌（LELC）的临床病理学特点、诊断、鉴别诊断及预后。 方法： 分析2008年1月至2018年12月台州恩泽医疗中心（集团）浙江省台州医院收集的21例LELC的临床资料、病理特点、免疫组织化学染色及原位杂交结果，并复习相关文献。 结果： 21例LELC中，发生于涎腺8例、胃5例、肺6例、乳腺1例、宫颈1例。HE染色显微镜下形态相似，在丰富的淋巴细胞背景中见散在分布低分化或未分化的癌细胞，呈巢片状排列，免疫组织化学显示癌细胞表达广谱细胞角蛋白（CKpan）、p63、p40，间质反应性淋巴细胞表达CD8、CD20。原位杂交EB病毒编码的小RNA（EBER，16/21）阳性。 结论： LELC是少见的上皮性恶性肿瘤，大部分与EB病毒感染相关，具有独特的临床病理学特征，预后相对较好。.

Lymphoepithelioma-like carcinoma associated to EPSTEIN-BARR virus. An extremely rare cause of tumor in duodenum

Background: Lymphoepithelioma-like carcinoma is a very rare disease in digestive tube. Duodenum is a extremely rare location for this tumor. Usually, it is associated to Epstein-Barr virus infection. The aim is to present a case of lymphoepithelioma-like carcinoma in duodenum with a bibliographic review to know its management. Material and Methods: A 45 years-old male consulted for epigastric pain of 3 months evolution. Gastroscopy study found a tumor in third portion of the duodenum. Histological study showed a carcinoma poorly differentiated. A CT study was performed, finding a stenosis in third duodenal part. Results: The patient underwent surgery with resection of third and fourth portion of the duodenum. Reconstruction was with a duodenum-jejunostomy (anastomosis of second part of duodenum and jejunum Roux-en-Y). Histological study of specimen showed an epithelial tumor (carcinoma) with Epstein-Barr virus infection. A carcinoma displaying undifferentiated features with dense lymphoplasmacytic infiltration was defined as a lymphoepithelioma-like. In four years of follow-up, there is not recurrence of the tumor. Conclusions: Lymphoepithelioma-like carcinoma is a very rare cause of tumor in duodenum.

Atezolizumab in a CoHort of pretreated, advanced, non-small cell lung cancer patients with rare HistologiCal SubtypEs (CHANCE trial).

Background:Although immunotherapy with immune-checkpoint inhibitors (ICIs) has profoundly changed the therapeutic scenario in the treatment of advanced non-small cell lung cancer (NSCLC), trials of ICIs only enrolled NSCLC patients with common histology. Atezolizumab was approved by the United States Food and Drug Administration (US FDA) in October 2016 and by the European Medicines Agency (EMA) in September 2017 for the treatment of patients with metastatic NSCLC whose disease progressed during or following platinum-containing chemotherapy, regardless of PD-L1 expression.Methods:We designed a single-arm, multicenter, two-stage phase II study and plan to enroll 43 patients. The primary objective of the study is to evaluate the antitumor activity of atezolizumab in advanced NSCLC patients with rare histology subtypes. Patients with prior atezolizumab or ICI treatment and with untreated, symptomatic, or progressing brain metastases will be excluded. The primary endpoint is disease control rate. Secondary objectives are toxicity and safety, overall response rate, progression-free survival, overall survival, and time to progression. Diagnosis of NSCLC with rare histology will be confirmed by central pathology revision, and will include: colloid carcinoma, fetal adenocarcinoma, non-endocrine large cell carcinoma, sarcomatoid carcinoma, salivary gland-type tumor, lymphoepithelioma-like carcinoma, and NUT-nuclear protein in testis carcinoma. Archival tumor tissue is required for correlative studies of PD-L1 expression on tumor cells and tumor infiltrating lymphocytes.Conclusions:Therapeutic options in NSCLC with rare histology subtypes, to be assessed in specifically designed trials, are an unmet need. This trial will help elucidate the role of atezolizumab as a viable option in this setting.

Lymphoepithelioma-like carcinoma of the duodenum: a doubly infrequent tumor

Lymphoepithelioma-like carcinoma of the duodenum: a doubly infrequent tumor

A Case of Lymphoepithelioma-like Carcinoma in the Breast

A Case of Lymphoepithelioma-like Carcinoma in the Breast