Primary extranodal natural killer/T-cell lymphoma of bronchus and lung: A case report and review of literature.
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ABSTRACT:
SUBMITTER: Chien CC
PROVIDER: S-EPMC4718123 | biostudies-other | 2016 Jan
REPOSITORIES: biostudies-other
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Project description:We described a 56-year-old Indonesian man with one-month history of nasal obstruction and rapidly increasing nasal swelling and two weeks history of severe facial pain with foul smelling discharge, bleeding and noticing live maggots emerging from his nasal and oral cavity. On examination, he appeared cachexic with a markedly swollen, erythematous and deformed external nose. Live maggots, pus and necrotic tissues were found in both nasal cavities, with erosion of upper gingiva and hard palate. Patient was managed initially with tracheostomy under local anaesthesia, followed by removal of around 350 live maggots from the nasal cavities and debridement of necrotic tissues. A midline nasal cavity mass which extended laterally into the nasal cavity was found, along with a large defect over the gingival labial sulcus with necrotic hard palate and a communication between oral and nasal cavities. Biopsies were taken from the nasal mass, gingiva and hard palate. Histopathological results from the biopsies showed diffuse, aggressive infiltrative malignant lymphoid cells with widespread angionecrosis, consistent with features of extranodal NK/T-cell lymphoma. To our knowledge, there is only one other reported case where the diagnosis of ENKTCL was made after patient presented with oro-nasal myiasis. Clinicians should have raised awareness on this atypical presentation so that further investigation and management can be implemented promptly.
| S-EPMC8977926 | biostudies-literature
Project description:IntroductionPyoderma gangrenosum (PG) is a neutrophilic dermatosis that may be associated with systemic diseases. The association of PG with lymphoid malignancies has rarely been reported. Extranodal natural killer/T-cell lymphoma (ENKTL) is a rare but aggressive entity with a poor prognosis. Here, we report the case of a patient who had idiopathic PG refractory to systemic steroids and subsequently developed ENKTL.Case reportA 70-year-old man presented with a 2-month history of intermittent fever and multifocal painful papules, plaques, and ulcerations on his extremities. The histological and culture results of the lesions were consistent with those of PG. A thorough work-up was performed and did not demonstrate any underlying systemic diseases including malignancy. The PG lesions were refractory to systemic steroid therapy. An enlarging nodule was observed over his right infraorbital area 4 months after the onset of the skin eruptions. The nodule was later biopsied and diagnosed as ENKTL by using histopathological and immunohistochemical studies. Fludeoxyglucose positron emission tomography/computed tomography revealed multiple intense fludeoxyglucose-avid masses in the bones and lungs, suggesting multiorgan metastases. The patient rejected chemotherapy and died 4 weeks after the diagnosis.ConclusionThe present case indicates that in any patient with idiopathic PG refractory to conventional therapy, the presence of any underlying disease or malignancy must be thoroughly evaluated. The present case serves as a reminder that when assessing patients with PG, clinicians should increase their awareness regarding the delayed association with malignancy, even in the absence of a concomitant systemic disease at presentation. Furthermore, the prompt evaluation of any suspicious lesions in the context of PG for the possibility of a malignant nature can improve the prognosis, particularly in cases of aggressive malignancy. Understanding the cutaneous spectrum of ENKTL is crucial because of its variable clinical appearance and aggressive nature. Our case demonstrates that PG can be a presenting sign of ENKTL.
| S-EPMC5059062 | biostudies-literature
Project description:BackgroundExtranodal NK/T-cell lymphoma (ENKTL) is an aggressive non-Hodgkin lymphoma that typically develops in the upper aerodigestive tract.Case presentationWe encountered an ENKTL patient who presented with purpura-like rashes and foot drops as initial symptoms and later developed other peripheral nerve involvement. The nerve conduction study of both the motor nerve and the sensory nerve showed axonal damage resembling mononeuropathy multiplex. Although the initial response to steroids was encouraging, the patient's symptoms reappeared and aggravated. A biopsy of the abdominal subcutaneous fat tissue with additional immunohistochemistry revealed neoplastic NK/T lymphocytes.ConclusionWe reported the first case presented as mononeuropathy multiplex as the initial clinical manifestation in ENKTL patients. Lymphoma should be considered in the diagnosis of atypical mononeuropathy in multiplex patients.
| S-EPMC10702496 | biostudies-literature
Project description:PurposeTo investigate the clinicopathological features, survival and prognostic factors of primary intestinal extranodal natural killer/T-cell lymphoma, nasal type (PI-ENKTCL).MethodsClinical and histological characteristics of PI-ENKTCL cases were retrospectively evaluated. Immunohistochemical phenotype and status of Epstein-Barr virus (EBV) and T-cell receptor (TCR) gene rearrangement were examined. The overall survival and prognostic parameters were also analyzed.ResultsFifty-five (2.7%) cases with PI-ENKTCL were identified out of 2017 archived ENKTCL cases, with a median age of 39 years and a male to female ratio of 2.1:1. The most common symptom was abdominal pain (90.9%), accompanied frequently with fever and less commonly with intestinal perforation or B symptoms. Small intestine (50.9%) was the most common site to be involved. 47.3% and 36.4% cases presented with stage I and II diseases, respectively. Histologically, most cases displayed characteristic morphologic changes of ENKTCL. Cytoplasmic CD3, TIA-1 and CD56 expression was found in 100%, 94.5% and 89.1% of cases, respectively. In situ hybridization detection for EBV demonstrated positive results in all cases. Monoclonal TCR gene rearrangement was found in 52.9% of tested cases. Chemotherapy with a DICE or L-asparaginase/peg-asparginase-containing regimen was most often employed. Both advanced tumor stage and B symptoms were independent inferior prognostic factors (p = 0.001 and p = 0.010). Noticeably, 6 cases demonstrated a CD4-positive phenotype. These cases featured a relatively older median age (58 years), predominance of small/medium-sized neoplastic cells, a higher rate of TCR rearrangement and slightly favorable outcome.ConclusionWe reported by far the largest series of PI-ENKTCL, and demonstrated its heterogeneity, aggressive clinical behavior and unsatisfying response to the current therapeutic strategies. Those CD4-positive cases might represent a unique subtype of PI-ENKTCL or distinct entity. Further investigations are required for the better understanding and management of this unusual disease.
| S-EPMC5001693 | biostudies-literature
Project description:Extranodal natural killer/T-cell lymphoma, nasal type (ENKTL) is a rare subtype of lymphoma that is often associated with poor clinical prognosis. Several studies have shown that hepatitis B virus (HBV) infection may be associated with increased risk of B-cell non-Hodgkin lymphoma; however, because of the rarity of ENKTL, little is known about its association with HBV. Our study aimed to assess whether HBV infection was associated with increased odds of ENKTL. We conducted a hospital-based case-control study including 417 ENKTL cases and 488 age- and sex-matched subjects with nonmalignant diseases unrelated to HBV infection. Multivariable unconditional logistic regression analyses were performed to estimate adjusted odds ratios [AOR] and their corresponding 95% confidence intervals (CI). The results of the multivariable analysis showed that after adjustment for a set of known risk factors, patients previously infected with HBV (HBsAg-seronegative/anti-HBc-seropositive) and naturally immune to HBV (anti-HBs-seropositive/anti-HBc-seropositive) were at significantly greater odds of being diagnosed with ENKTL (AOR, 1.497; 95% CI 1.098-2.042, P=0.033 and AOR, 1.871; 95% CI 1.302-2.689, P=0.001, respectively). After adjusting for other factors, significantly greater odds of being diagnosed with ENKTL were observed among cases who reported ever drinking alcohol (AOR, 1.675; 95% CI 1.054-2.660, P=0.029). The odds of ENKTL diagnosis were not significantly associated with ABO blood type, cigarette smoking status or family history of cancer. The results of our study suggest that patients previously infected with HBV and naturally immune to HBV were at greater odds of being diagnosed with ENKTL.
| S-EPMC5604198 | biostudies-literature
Project description:Natural killer/T-cell (NK/T) lymphomas represent a group of rare tumors of NK and NK-T cells. The World Health Organization classifies NK-cell tumors into three types, extranodal NK/T-cell lymphomas (ENKL, nasal and non-nasal), NK-cell leukemias, and a blastic variant (CD4-positive, CD56-positive hematodermic neoplasms). We focus our review to the current concepts in biology and treatment of ENKL. Though considerable advances have been made in our understanding of NK-cell biology, malignant transformation including the role of Epstein-Barr virus, and prognosis, the rare nature of ENKL and its heterogeneity limit the ability to standardize therapy. Radiotherapy is fundamental to treatment of early-stage disease with a role for chemoradiotherapy among high-risk patients. The clinical course of advanced disease is highly aggressive with frequent chemotherapy resistance and a poor prognosis. Therapeutic approaches to advanced-stage or relapsed and refractory disease, including the appropriate sequence of chemotherapy, combined modality therapy, and stem cell transplantation is not well-established. International and multicenter clinical trials are needed for this rare and aggressive disease.
| S-EPMC5568825 | biostudies-other
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 ?? 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.
| S-EPMC6476925 | biostudies-literature
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).
| S-EPMC6790270 | biostudies-literature
Project description:Extranodal natural killer/T-cell lymphoma (ENKTL) is an aggressive, rare lymphoma of natural killer (NK) cell origin with poor clinical outcomes. Here we used phenotypic and molecular profiling, including epigenetic analyses, to investigate how ENKTL ontogeny relates to normal NK-cell development. We demonstrate that neoplastic NK cells are stably, but reversibly, arrested at earlier stages of NK-cell maturation. Genes downregulated in the most epigenetic immature tumors were associated with polycomb silencing along with genomic gain and overexpression of EZH2. ENKTL cells exhibited genome-wide DNA hypermethylation. Tumor-specific DNA methylation gains were associated with polycomb-marked regions, involving extensive gene silencing and loss of transcription factor binding. To investigate therapeutic targeting, we treated novel patient-derived xenograft (PDX) models of ENKTL with the DNA hypomethylating agent, 5-azacytidine. Treatment led to reexpression of NK-cell developmental genes, phenotypic NK-cell differentiation, and prolongation of survival. These studies lay the foundation for epigenetic-directed therapy in ENKTL.SignificanceThrough epigenetic and transcriptomic analyses of ENKTL, a rare, aggressive malignancy, along with normal NK-cell developmental intermediates, we identified that extreme DNA hypermethylation targets genes required for NK-cell development. Disrupting this epigenetic blockade in novel PDX models led to ENKTL differentiation and improved survival. This article is highlighted in the In This Issue feature, p. 85.
| S-EPMC9414823 | biostudies-literature
Project description:BackgroundDue 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.MethodsWe 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.ResultsThe 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.ConclusionsPulmonary 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.
| S-EPMC10722052 | biostudies-literature