The Pathologic and Genetic Characteristics of Extranodal NK/T-Cell Lymphoma

Kim, Hyunsung; Ko, Young Hyeh

doi:10.3390/life12010073

Cited by 17 publications

(33 citation statements)

References 156 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A common immunophenotype of NK-cell lineage ENKL is the presence of CD2, CD56, cytoplasmatic CD3 and cytotoxic markers (TIA-1, perforin and granzyme B) with the absence of surface CD3. Less frequently, the lymphoma cells originate from T cells and show a different phenotype often characterised by the presence of surface CD3, CD2, CD8 and/or CD7 1–5 9. ENKL has a strong association with EBV.…”

Section: Discussionmentioning

confidence: 99%

Ptosis, facial numbness and parotid gland mass as the first symptoms of an extranodal NK/T cell lymphoma, nasal type

et al. 2022

View full text Add to dashboard Cite

We present a case of a patient visiting the ear, nose and throat department with a parotid gland mass, ptosis and facial numbness. CT imaging confirmed a mass in the parotid gland; however, it also revealed a mass in the left maxillary sinus. MRI, positron emission tomography combined with CT and nasal biopsy confirmed the diagnosis of a extranodal natural killer/T cell lymphoma, nasal type. Because this is a rare clinical entity in Western society, patients are typically diagnosed in an advanced stage; symptoms resemble chronic rhinosinusitis and histopathological analysis is challenging. In this atypical case, the patient presented with symptoms of ptosis, parotid gland mass and facial numbness instead of nasal symptoms. In this case, we want to emphasise that diagnosing a sinonasal NK/T-cell lymphoma is often challenging.

show abstract

Section: Discussionmentioning

confidence: 99%

Ptosis, facial numbness and parotid gland mass as the first symptoms of an extranodal NK/T cell lymphoma, nasal type

et al. 2022

View full text Add to dashboard Cite

show abstract

“…Epigenetic modifiers, including MLL2 , MLL3 , BCOR , TET2 , EP300 , and ARID1A , are mutated in about 24.8% of cases with ENKTCL ( 9 ). Accordingly, epigenetic regulation is an important treatment strategy for this disease.…”

Section: Treatment Of Advanced-stage and R/r Enktclmentioning

confidence: 99%

“…Moreover, common genetic aberrations (18.2%) in ENKTCL involve genes related to the JAK-STAT pathway ( JAK3 , JAK2 , STAT3 , STAT5B , SOCS1 , and PTPRK ). JAK or STAT activating mutations lead to constitutive activation of the JAK-SATAT pathway, which plays a major role in ENKTCL cell growth and survival ( 9 , 201 ). These findings make JAK an interesting target for the treatment of ENKTC.…”

Section: Treatment Of Advanced-stage and R/r Enktclmentioning

confidence: 99%

“…In particular, we mainly focused on several neglected or unanswered issues in the treatment of ENKTCL, including the optimal staging method, the best partner of Asp, the individualized administration of Asp, the preferred sequence of CT and RT and so on. The pathologic and genetic features of this disease have recently been well reviewed by others (8)(9)(10). When analyzing the outcomes of patients treated with different approaches or modalities, we visualized the data using figures, as most data are from retrospective studies with small cohorts and heterogenous treatments, which are difficult to compare directly.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Treatment of extranodal NK/T-cell lymphoma: From past to future

et al. 2023

View full text Add to dashboard Cite

Extranodal NK/T-cell lymphoma (ENKTCL) is the most common subtype of T/NK-cell lymphoma in Asia and Latin America, but very rare in North American and Europe. Patient survival has improved significantly over the past two decades. However, standard treatment has not yet been established, although dozens of prospective trials have been conducted. To help understand how the treatment of ENKTCL has evolved in the past and what trends lie ahead, we have comprehensively reviewed the treatment of this aggressive malignancy, with a particular focus on neglected or unanswered issues, such as the optimal staging method, the best partner of asparaginase (Asp), the individualized administration of Asp, the preferred sequence of CT and RT and so on. Overall, the 5-year overall survival (OS) of patients with Ann Arbor stage I/II disease increased from < 50% in the early 20th century to > 80% in recent years, and the median OS of patients with Ann Arbor stage III/IV disease increased from < 1 year to more than 3 years. The improvement in patient survival is largely attributable to advances in radiation technology and the introduction of Asp and anti-PD-1/PD-L1 immunotherapy into practice. Radiotherapy is essential for patients with early-stage disease, while Asp-based chemotherapy (CT) and PD-1/PD-L1 inhibitors significantly improved the prognosis of patients with advanced-stage disease. ENKTCL management is trending toward simpler regimens, less toxicity, and higher efficacy. Novel drugs, such as manufactured T cells, monoclonal antibodies, and small molecule inhibitors, are being intensively investigated. Based on the fact that ENKTCL is highly resistant to cytotoxic drugs except Asp, and aggressive CT leads to higher toxicity rather than better outcomes, we recommend it is unnecessary to expend additional resources to compare different combinations of Asp with cytotoxic agents. Instead, more efforts should be made to optimize the use of Asp and immunotherapy to maximize efficacy and minimize toxicity, explore ways to overcome resistance to Asp and immunotherapy, identify novel treatment targets, and define subpopulations who may benefit more from specific treatments.

show abstract

“…Interestingly, EBV may infect not only B-lymphocytes but even T-lymphocytes and NK-cells, although the mechanism for virus entrance into T/NK-cells is not well-known [ 34 , 35 , 36 , 37 , 38 ].…”

Section: Overview On Ebv Biologymentioning

confidence: 99%

Intravascular NK/T-Cell Lymphoma: What We Know about This Diagnostically Challenging, Aggressive Disease

Zanelli

Parente

Sanguedolce

et al. 2022

Cancers

View full text Add to dashboard Cite

Intravascular lymphoma is a form of lymphoid malignancy characterized by neoplastic cells growing almost exclusively within the lumina of small- to medium-sized blood vessels. Most cases are of B-cell origin with rare cases of natural killer or T-cell lineage. Extranodal sites are affected, mainly the skin and central nervous system, although any organ may be involved. Intravascular NK/T-cell lymphoma deserves special attention because of its clinicopathologic features and the need for adequate immunophenotyping combined with clonality test for a proper diagnosis. Moreover, intravascular NK/T-cell lymphoma is strongly linked to Epstein–Barr virus (EBV), which is considered to play a role in tumorigenesis and to be responsible for the aggressive behavior of the disease. In this paper, we review the current knowledge on this rare lymphoma and, in particular, the most recent advances about its molecular landscape. The main distinguishing features with other EBV-related entities, such as extranodal NK/T-cell lymphoma, EBV-positive primary nodal T/NK-cell lymphoma, and aggressive NK-cell leukemia, are discussed to help pathologists obtain the correct diagnosis and consequently develop an adequate and prompt therapy response.

show abstract

The Pathologic and Genetic Characteristics of Extranodal NK/T-Cell Lymphoma

Cited by 17 publications

References 156 publications

Ptosis, facial numbness and parotid gland mass as the first symptoms of an extranodal NK/T cell lymphoma, nasal type

Ptosis, facial numbness and parotid gland mass as the first symptoms of an extranodal NK/T cell lymphoma, nasal type

Treatment of extranodal NK/T-cell lymphoma: From past to future

Intravascular NK/T-Cell Lymphoma: What We Know about This Diagnostically Challenging, Aggressive Disease

Contact Info

Product

Resources

About