Dataset Information

Pulmonary extranodal natural killer/T-cell lymphoma: clinical presentation, CT characteristics, and patient outcomes.

ABSTRACT:

Background

Due to the extreme rarity of pulmonary extranodal natural killer/T-cell lymphoma (ENKTCL), studies on this lymphoma type are limited. We aimed to investigate the clinical presentations, computed tomography (CT) findings, CT dynamic changes, and outcomes of patients diagnosed with pulmonary ENKTCL.

Methods

We conducted a retrospective cohort study on ENKTCL, nasal type, at West China Hospital, from January 2010 to January 2023. Out of 27 initial cases with pulmonary ENKTCL, we excluded 4 due to the lack of chest CT images, resulting in a final cohort of 23 patients. Our evaluation covered clinical features, laboratory findings, CT presentations, treatment strategies, and survival outcomes. Survival analyses were performed using the Kaplan-Meier method, with log-rank tests for survival curve comparisons. Given the small sample size, our interpretation of the data is mainly descriptive.

Results

The most common CT presentations in our institution was solitary or multiple nodules (7/23, 30.4%). The halo sign (78.3%) and floating vessels sign (83.3%) were commonly observed in pulmonary ENKTCL. Elevated lactate dehydrogenase (LDH) levels were observed in 20 (86.9%) patients. The median overall survival (OS) for pulmonary ENKTCL patients was 1 month; 3 cases displayed a favorable prognosis, maintaining complete remission (CR) status for 1.5-7 years after receiving chemotherapy. Chemotherapy led to improved prognosis, with a median survival of 5 months seen in patients who received chemotherapy. Patients with bilateral lung involvement had worse outcomes compared to those with unilateral lesions.

Conclusions

Pulmonary ENKTCL is a rare and aggressive disease, often misdiagnosed due to nonspecific clinical and CT features. Laboratory tests, failure of antibiotic treatment, and "floating vessels sign" on enhanced CT scans may aid in diagnosis. Timely chemotherapy may improve survival, emphasizing the importance of early detection and prompt treatment.

SUBMITTER: Li S

PROVIDER: S-EPMC10722052 | biostudies-literature | 2023 Dec

REPOSITORIES: biostudies-literature

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Publications

Pulmonary extranodal natural killer/T-cell lymphoma: clinical presentation, CT characteristics, and patient outcomes.

Li Shuang S Fang Xin X Liu Ying Y Yue Qiang Q Yu Jianqun J

Quantitative imaging in medicine and surgery 20231109 12

<h4>Background</h4>Due to the extreme rarity of pulmonary extranodal natural killer/T-cell lymphoma (ENKTCL), studies on this lymphoma type are limited. We aimed to investigate the clinical presentations, computed tomography (CT) findings, CT dynamic changes, and outcomes of patients diagnosed with pulmonary ENKTCL.<h4>Methods</h4>We conducted a retrospective cohort study on ENKTCL, nasal type, at West China Hospital, from January 2010 to January 2023. Out of 27 initial cases with pulmonary ENKT ...[more]

PMID: 38106312

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Project description:Extranodal natural killer (NK)/T-cell lymphoma, nasal type (NNKTL) has very unique epidemiological, etiologic, histologic, and clinical characteristics. It is commonly observed in Eastern Asia, but quite rare in the United States and Europe. The progressive necrotic lesions mainly in the nasal cavity, poor prognosis caused by rapid local progression with distant metastases, and angiocentric and polymorphous lymphoreticular infiltrates are the main clinical and histologic features. Phenotypic and genotypic studies revealed that the lymphoma is originated from either NK- or γδ T-cell, both of which express CD56. In 1990, the authors first reported the presence of Epstein-Barr virus (EBV)-DNA and EBV-oncogenic proteins, and EBV has now been recognized to play an etiological role in NNKTL. in vitro studies revealed that a wide variety of cytokines, chemokines, and micro RNAs, which may be produced by EBV-oncogenic proteins in the lymphoma cells, play important roles for tumor progression in NNKTL, and could be therapeutic targets. In addition, it was revealed that the interaction between NNKTL cells and immune cells such as monocytes and macrophages in NNKTL tissues contribute to lymphoma progression. For diagnosis, monitoring the clinical course and predicting prognosis, the measurements of EBV-DNAs and EBV-micro RNAs in sera are very useful. For treatment with early stage, novel concomitant chemoradiotherapy such as DeVIC regimen with local radiotherapy and MPVIC-P regimen using intra-arterial infusion developed with concomitant radiotherapy and the prognosis became noticeably better. However, the prognosis of patients with advanced stage was still poor. Establishment of novel treatments such as the usage of immune checkpoint inhibitor or peptide vaccine with molecular targeting therapy will be necessary. This review addresses recent advances in the molecular understanding of NNKTL to establish novel treatments, in addition to the epidemiologic, clinical, pathological, and EBV features.

Project description:BackgroundExtranodal natural killer T-cell lymphoma (NKTCL), nasal type, is a rare and aggressive malignancy that occurs predominantly in Asian and Latin American populations. Although Epstein-Barr virus infection is a known risk factor, other risk factors and the pathogenesis of NKTCL are not well understood. We aimed to identify common genetic variants affecting individual risk of NKTCL.MethodsWe did a genome-wide association study of 189 patients with extranodal NKTCL, nasal type (WHO classification criteria; cases) and 957 controls from Guangdong province, southern China. We validated our findings in four independent case-control series, including 75 cases from Guangdong province and 296 controls from Hong Kong, 65 cases and 983 controls from Guangdong province, 125 cases and 1110 controls from Beijing (northern China), and 60 cases and 2476 controls from Singapore. We used imputation and conditional logistic regression analyses to fine-map the associations. We also did a meta-analysis of the replication series and of the entire dataset.FindingsAssociations exceeding the genome-wide significance threshold (p<5 × 10(-8)) were seen at 51 single-nucleotide polymorphisms (SNPs) mapping to the class II MHC region on chromosome 6, with rs9277378 (located in HLA-DPB1) having the strongest association with NKTCL susceptibility (p=4·21 × 10(-19), odds ratio [OR] 1·84 [95% CI 1·61-2·11] in meta-analysis of entire dataset). Imputation-based fine-mapping across the class II MHC region suggests that four aminoacid residues (Gly84-Gly85-Pro86-Met87) in near-complete linkage disequilibrium at the edge of the peptide-binding groove of HLA-DPB1 could account for most of the association between the rs9277378*A risk allele and NKTCL susceptibility (OR 2·38, p value for haplotype 2·32 × 10(-14)). This association is distinct from MHC associations with Epstein-Barr virus infection.InterpretationTo our knowledge, this is the first time that a genetic variant conferring an NKTCL risk is noted at genome-wide significance. This finding underlines the importance of HLA-DP antigen presentation in the pathogenesis of NKTCL.FundingTop-Notch Young Talents Program of China, Special Support Program of Guangdong, Specialized Research Fund for the Doctoral Program of Higher Education (20110171120099), Program for New Century Excellent Talents in University (NCET-11-0529), National Medical Research Council of Singapore (TCR12DEC005), Tanoto Foundation Professorship in Medical Oncology, New Century Foundation Limited, Ling Foundation, Singapore National Cancer Centre Research Fund, and the US National Institutes of Health (1R01AR062886, 5U01GM092691-04, and 1R01AR063759-01A1).

Dataset Information

Pulmonary extranodal natural killer/T-cell lymphoma: clinical presentation, CT characteristics, and patient outcomes.

Background

Methods

Results

Conclusions

Publications

Pulmonary extranodal natural killer/T-cell lymphoma: clinical presentation, CT characteristics, and patient outcomes.

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