Pulmonary lymphoepithelioma‐like carcinoma with echinoderm microtubule‐associated protein‐like 4‐anaplastic lymphoma kinase (EML4‐ALK) fusion gene

Ose, Naoko; Kawai, T; Ishida, Daisuke; Kobori, Yuko; Takeuchi, Yukiyasu; Senba, Hidetoshi

doi:10.1002/rcr2.200

Cited by 8 publications

(5 citation statements)

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“…In our study, 25 participants had undergone gene testing and only 2 had EGFR mutations, suggesting that EGFR may not play an important role in the carcinogenesis of LELC. Rearrangement of the ALK gene is another driver oncogene in lung cancer and is rarely found in LELC (20,21), only 1 case in Japan has reported EML4-ALK fusion gene in lung LELCs (22). Similarly, no patients with the ALK fusion gene mutation were identified in our study.…”

Section: Discussioncontrasting

confidence: 43%

Clinical observation of pulmonary lymphoepithelioma-like carcinoma

Zhou¹,

Wei²,

Gao³

et al. 2021

J Thorac Dis

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Background: Lymphoepithelioma-like carcinoma (LELC) of the lung is a rare type of non-small cell lung cancer (NSCLC), and researches of it are still not enough.Methods: In this study, we retrospectively analyzed 36 patients with LELC diagnosed in the Fifth Affiliated Hospital of Sun Yat-sen University and Zhaoqing First People's Hospital from January 2014 to June 2021, to investigate the clinical manifestations, tumor markers, treatment, and prognosis of LELC. Clinical data including age, gender, smoking history, family history of cancers, Epstein-Barr virus (EBV) encoding RNA (EBER) status, gene mutations, programmed death-ligand 1 (PD-L1) expression, treatment, and prognosis.Results: There was a total of 36 participants in this study, 16 males and 20 females, the median age was 57 years (37-76 years). A total of 22 cases (61.1%) were advanced (stage III and IV), and EBER was 94.4% positive. Most patients were treated with surgery, platinum chemotherapy, or radiotherapy. At the time of 31 June 2021, 33 participants had survived, and the longest survival time was 72 months. Lung LELC was more common in old participants (≥59 years) and was not associated with smoking history. Expression of PD-L1 was positive in the majority (27 cases, 75%) and participants with positive PD-L1 expression tended to have longer progression-free survival (PFS) and overall survival (OS) time than those with negative PD-L1 expression. Conclusions: Pulmonary LELC usually occurs innon-smoking patients and is associated with EBV infection. Common treatments for tumors include multimodal therapy. The expression of PD-1 may be related to the prognosis of LELC, but more studies are needed to support further optimization of the treatment of LELC.

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Section: Discussioncontrasting

confidence: 43%

Clinical observation of pulmonary lymphoepithelioma-like carcinoma

Zhou¹,

Wei²,

Gao³

et al. 2021

J Thorac Dis

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“…ALK alteration was detected positive in three PLELC patients in our study, while only one was reported in the literature by Ose et al 38 The variant rates may be due to the small sample size of each study which warrants further investigation. In the study by Gou and Wu which demonstrated the frequencies of driver mutations in nonsmall cell lung cancer in China, 19 the mutation rates of EGFR were 48.4% (675/1795), 4.3% (9/208), and 28.2% (570/2021) in lung adenocarcinoma, squamous cell cancer 36 Hong Kong, China 11 NA 0 NA NA Liang et al 8 Guangzhou, China 52 0/11 NA NA NA Liu et al 29 Guangzhou, China 32 0 NA NA NA Wang et al 28 Guangzhou, China 42 1 0 NA NA Fang et al 37 Guangzhou, China 113 2 0 NA NA Chang et al 30 Tai Wan, China 66 8 0 0 0 (BRAF) Ose et al 38 Osaka, Japan 1 0 1 NA NA Hong et al 31 Guangzhou, China 91 0 0 NA 14 (P53) Lin et al 39 Guangzhou, China 39 0/19 NA NA NA Lin et al 40 Fuzhou, China 20 0/3 0/2 0/1 NA Xie et al 10 Guangzhou, China 429 2 0 0 NA Zhou et al 41 Guangzhou, China 27 0 0 1 7 (P53) Yin et al 42 Guangzhou 43 An et al reported that the mutation rate of driver genes might differ among different histology subtypes and smoking status. 44 EGFR and ALK alteration is rare in PLELC.…”

Section: Discussioncontrasting

confidence: 67%

Low frequency of mutation of epidermal growth factor receptor (EGFR) and arrangement of anaplastic lymphoma kinase (ALK) in primary pulmonary lymphoepithelioma‐like carcinoma

Yin

Feng

et al. 2019

Thoracic Cancer

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Background: Primary pulmonary lymphoepithelioma-like carcinoma (PLELC) is a rare and unique subtype of lung cancer. However, the prevalence of driver alterations, such as epidermal growth factor receptor (EGFR) mutations and anaplastic lymphoma kinase (ALK) rearrangements, and the response of tyrosine kinase inhibitor (TKIs) in PLELC has not been thoroughly investigated. Method: We retrospectively reviewed the genetic profiles and treatment course of 330 PLELC patients at the Guangdong Lung Cancer Institute (GLCI) from based on their mention of "driver mutations" and "the response of TKIs to mutant PLELC". Results: Genetic alterations of EGFR/ALK were tested in 203 patients (203/330, 61.5%). Five patients (5/175, 2.9%) had EGFR mutation and three patients (3/140, 2.1%) had ALK alteration. From the total of 15 articles identified from electronic searches, 1071 PLELC cases mentioned the driver mutations. EGFR mutation and ALK rearrangement were detected in 15 patients and one patient, respectively. In total, there were four EGFR/ALK mutant PLELC patients who received targeted therapy as palliative treatment at the GLCI and in the literature. However, there was disease progression in all cases one month after use of TKIs. Conclusion: The mutation rates of EGFR and ALK were low in PLELC. EGFR and ALK TKIs showed limited response in EGFR/ALK mutant PLELC. Further studies are needed to explore other molecular targets to optimize the therapeutic strategy for PLELC. Key points• The mutation rates of EGFR and ALK in PLELC were lower than other common types of non-small cell lung cancer. • EGFR/ALK TKIs showed a limited response in EGFR/ALK mutant PLELC.• This large cohort study demonstrates the prevalence of EGFR mutation and ALK alteration, and summarizes the response of targeted therapy in mutant PLELC patients.

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“…Solid and off-white tumors with large nuclei cells in nets infiltrated by massive lymphocytes were stable findings for the diagnosis of LELC [27]. What’s more, a detailed systemically physical examination and necessary radiologic tests to exclude a possible extrapulmonary metastatic LELC, especial nasopharyngeal carcinoma, are also required before making a primary pulmonary LELC diagnosis [28, 29].…”

Section: Discussionmentioning

confidence: 99%

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

Chen

Zhou

et al. 2019

Respir Res

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BackgroundPrimary 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.MethodsForty-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.ResultsIn 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.ConclusionPulmonary 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.

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Pulmonary lymphoepithelioma‐like carcinoma with echinoderm microtubule‐associated protein‐like 4‐anaplastic lymphoma kinase (EML4‐ALK) fusion gene

Cited by 8 publications

References 4 publications

Clinical observation of pulmonary lymphoepithelioma-like carcinoma

Clinical observation of pulmonary lymphoepithelioma-like carcinoma

Low frequency of mutation of epidermal growth factor receptor (EGFR) and arrangement of anaplastic lymphoma kinase (ALK) in primary pulmonary lymphoepithelioma‐like carcinoma

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

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