A 65-Year-Old Man With Persistent Cough and Large Nodular Opacity

Grimes, Brandon S.; Albores, J M

doi:10.1378/chest.14-1172

Cited by 3 publications

(5 citation statements)

References 5 publications

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“…PPLELC has obvious racial and geographical distribution characteristics. Among the 18 patients in our study, the male to female ratio was 4:5, the median age was 57 years, and the ratio of non-smokers to smokers was 2:1, which was consistent with previous studies ( Ho et al, 2000 ; Grimes et al, 2015 ). However, the epidemiological characteristics of patients in Western countries may differ from those of all Chinese cases.…”

Section: Discussionsupporting

confidence: 92%

“…EBV infection may be related to race and region. Almost all Asian patients have positive EBV detection, while most non-Asian patients showed negative results (Grimes et al, 2015). IHC markers are significant in pathological diagnosis, especially for PPLELC, since they are not solely based on the morphologic features.…”

Section: Discussionmentioning

confidence: 97%

“…EBV infection may be related to race and region. Almost all Asian patients have positive EBV detection, while most non-Asian patients showed negative results ( Grimes et al, 2015 ).…”

Section: Discussionmentioning

confidence: 99%

“…The majority of the patients with PPLELC do not have obvious clinical manifestations at the time of diagnosis ( Grimes et al, 2015 ), and the treatment modalities of PPLELC follow NSCLC regimens ( Ho et al, 2000 ; Ho et al, 2009 ). In addition, patients with advanced PPLELC are less likely to benefit from targeted therapy due to the low mutation rates of epidermal growth factor receptor (EGFR), anaplastic lymphoma kinase (ALK), and Kirsten rat sarcoma viral oncogene (KRAS), v-RAF murine sarcoma viral oncogene homolog B1 (BRAF), repressor of silencing 1 (ROS1) and protein 53 (P53) genes ( Chang et al, 2011 ; Liang et al, 2012 ; Chang et al, 2015 ).…”

Section: Introductionmentioning

confidence: 99%

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Molecular and Clinical Characteristics of Primary Pulmonary Lymphoepithelioma-Like Carcinoma

Fan

Shan

Gong

et al. 2021

Front. Mol. Biosci.

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Objectives: Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is an extremely rare subtype of non-small cell lung cancer (NSCLC). Currently, there are no established treatment protocols due to rarity of the cancer. Thus, this study aimed to explore the molecular and clinical characteristics of PPLELC.Study design and setting: Data from patients with PPLELC who were admitted to Zhejiang Cancer Hospital from August 2009 to September 2020 were retrospectively collected. Next-generation sequencing was performed to obtain a genomic profile and tumor mutation burden (TMB) value of patients with adequate tissue and divided them into two groups according to the expression level of PD-L1. The correlation of PD-L1 expression and the clinicopathological characteristics was evaluated by Pearson Chi-square test. Kaplan-Meier curves was applied to present the probability of survival between PD-L1 expression level and overall survival (OS). Moreover, the literature on the immunotherapy of advanced PPLELC published in PubMed between 2016 and 2020 were reviewed and the efficacy of immunotherapy were analyzed.Results: A total of 18 patients pathologically diagnosed as PPLELC were included. After a follow-up period of 8.8–138 months, 14 patients survived, three patients died and one patient lost, the median OS was 45.3 months Seven samples (tissue-available) tested by NGS and the median TMB was 2.5 mutations/Mb. 19 somatic mutated genes were recognized and TP53 (43%) and CYLD (43%) were the two most commonly mutated genes. Only seven patients who underwent NGS were tested for PD-L1. Three patients with high PD-L1 expression (PD-L1≥ 50%) and four patients with low PD-L1 expression (PD-L1 <50%) were included. No significant correlation was observed between PD-L1 expression and clinical characteristics (age, gender, smoking status, tumor stage, lymph node metastasis) (p > 0.05) and OS (p = 1). What’s more, 10 PPLELC patients involved in previous studies and one patient received nivolumab in the current study were collected retrospectively. 4/11 (36.4%) patients achieved PR, 6/11 (54.5%) patients achieved SD, and 1/11 (9.1%) patients achieved PD and the disease control rate (DCR) was 90.9%.Conclusions: The prognosis of PPLELC is better than that of other NSCLC, and immunotherapy may be a promising treatment to prolong the survival of advanced PPLELC patients. Whether the immunotherapy efficacy of PPLELC can be predicted by PD-L1 and TMB needs further clinical investigation. CYLD genetic alterations may participate in Epstein–Barr virus-mediated tumorigenesis in PPLELC, providing a novel therapeutic target.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 97%

“…EBV infection may be related to race and region. Almost all Asian patients have positive EBV detection, while most non-Asian patients showed negative results ( Grimes et al, 2015 ).…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Molecular and Clinical Characteristics of Primary Pulmonary Lymphoepithelioma-Like Carcinoma

Fan

Shan

Gong

et al. 2021

Front. Mol. Biosci.

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“…PPLELC, or LELC of the lungs, is classified as a subtype of non-small cell lung cancer (NSCLC) and is the most common type of LELC. Similar to NPC, it is found more commonly in Asians, in particular the Southern Chinese ( 36 – 38 ), and when found in westerners, it is typically EBV negative ( 39 – 41 ). It presents at a lower mean age of 51-55 years old and has a better prognosis than non-LELC lung cancers ( 42 ), with a higher incidence in non-smokers and Asian females ( 43 ).…”

Section: Introductionmentioning

confidence: 99%

Pathogenesis and therapeutic implications of EBV-associated epithelial cancers

Low,

Loh,

Peh

et al. 2023

Front. Oncol.

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Epstein-Barr virus (EBV), one of the most common human viruses, has been associated with both lymphoid and epithelial cancers. Undifferentiated nasopharyngeal carcinoma (NPC), EBV associated gastric cancer (EBVaGC) and lymphoepithelioma-like carcinoma (LELC) are amongst the few common epithelial cancers that EBV has been associated with. The pathogenesis of EBV-associated NPC has been well described, however, the same cannot be said for primary pulmonary LELC (PPLELC) owing to the rarity of the cancer. In this review, we outline the pathogenesis of EBV-associated NPC and EBVaGCs and their recent advances. By drawing on similarities between NPC and PPLELC, we then also postulated the pathogenesis of PPLELC. A deeper understanding about the pathogenesis of EBV enables us to postulate the pathogenesis of other EBV associated cancers such as PPLELC.

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Primary pulmonary lymphoepithelioma-like carcinoma

Fan

Qin

et al. 2020

Med Oncol

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A 65-Year-Old Man With Persistent Cough and Large Nodular Opacity

Cited by 3 publications

References 5 publications

Molecular and Clinical Characteristics of Primary Pulmonary Lymphoepithelioma-Like Carcinoma

Molecular and Clinical Characteristics of Primary Pulmonary Lymphoepithelioma-Like Carcinoma

Pathogenesis and therapeutic implications of EBV-associated epithelial cancers

Primary pulmonary lymphoepithelioma-like carcinoma

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