Extranodal Natural Killer/T-Cell Lymphoma (ENKTL) Malignant Pleural Effusion: A Rare Finding in an Uncommon Disease

May, Richard; Dinneen, Henry; Berman, Andrew

doi:10.1378/chest.1980642

Cited by 2 publications

(5 citation statements)

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“…To date, there are only a handful of reported cases with very limited data on the cytological features. [8][9][10][11][12][13][14][15][16][17] In this study of four new cases of effusion ENKTL, we found that the neoplastic cells were frequently of large to anaplastic morphology with azurophilic granules.…”

Section: Discussionmentioning

confidence: 99%

“…ENKTL rarely involves pleural effusion or ascites, which may occur at disease progression. In the literature, there are only a few cases of ENKTL with lymphomatous effusions . Herein we report four new cases of effusion ENKTL and the results of literature review, focusing on the cytopathological features, immunophenotype, and cellular lineage in terms of T‐vs NK‐cell origin.…”

Section: Introductionmentioning

confidence: 94%

“…We searched PubMed for cases of ENKTL-related malignant effusion as of July 2019 and selected only cases with relatively complete clinicopathological findings. [8][9][10][11][12][13][14][15][16][17]19,20 Four literature cases without confirmed EBV association were excluded. 8,11,17,19 We identified a total of 12 cases in the literatures.…”

Section: Literature Reviewmentioning

confidence: 99%

“…In the literature, there are only a few cases of ENKTL with lymphomatous effusions. [8][9][10][11][12][13][14][15][16][17] Herein we report four new cases of effusion ENKTL and the results of literature review, focusing on the cytopathological features, immunophenotype, and cellular lineage in terms of T-vs NK-cell origin. Interestingly, we found that effusion ENKTL was characterized by anaplastic large cell morphology, presence of cytoplasmic azurophilic granules, and a higher proportion of T-cell lineage as compared to de novo tumors arising from the prototypic sites.…”

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confidence: 99%

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Malignant effusions from extranodal NK/T‐cell lymphomas are frequently of anaplastic morphology with azurophilic granules and of T‐cell lineage

Liu

Chen

Chuang

2020

Diagnostic Cytopathology

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Background Extranodal natural killer/T‐cell lymphoma (ENKTL) nasal type is an extranodal lymphoma, with most cases arising from the prototypic nasal/upper aerodigestive tract and the cell of origin (COO) is frequently of NK cells. Effusion involvement by ENKTL is rare. The cytomorphological and immunophenotypical features and COO of effusion ENKTL are elusive. Design We report four new cases of effusion ENKTL, reviewing the cytomorphological features and investigating their COO by immunohistochemistry for T‐cell receptor (TCR) protein expression and clonality assay for TCR gene rearrangement. We also reviewed 12 additional cases of effusion ENKTL from the literature. Results All our four cases showed anaplastic tumor cells with fine to coarse azurophilic granules, with the primary tumor organs from the prototypic sites (2), small intestine (1), or indeterminate (1). The COO was either T‐cell (3) or NK‐cell (1). Compiling our four cases with that from the literature, all 16 effusion ENKTL cases were Epstein‐Barr virus‐associated and the majority (83%; 10/12) originated from the nonprototypic sites. Five of six (83%) effusion ENKTL cases were of T‐cell lineage. Prognosis was poor with a median survival time of only 2.4 months. Conclusions Our study revealed that ENKTL in the effusion might be characterized by anaplastic large cell morphology, cytoplasmic azurophilic granules, and a higher proportion of T‐cell lineage as compared to de novo tumors arising from the prototypic sites. ENKTL rarely presented in effusion, which is associated with dismal outcome. Large international studies are warranted to validate these findings.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 94%

Section: Literature Reviewmentioning

confidence: 99%

mentioning

confidence: 99%

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Malignant effusions from extranodal NK/T‐cell lymphomas are frequently of anaplastic morphology with azurophilic granules and of T‐cell lineage

Liu

Chen

Chuang

2020

Diagnostic Cytopathology

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“…Literature on cytological findings of ENKTL is scant, comprising mostly of single case reports . So far, only three cases have been diagnosed as ENKTL on fluid cytology . In the present study, we retrospectively analyse the cytological features of ENKTL in five histopathologically proven cases of ENKTL, in an attempt to identify any distinctive features that can aid in diagnosis.…”

Section: Introductionmentioning

confidence: 99%

Spectrum of cytomorphological features of extranodal NK/T‐cell lymphoma, nasal type

et al. 2019

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Objective: Extranodal natural killer/T-cell lymphoma, nasal type (ENKTL) is an aggressive extranodal lymphoma of NK-cell or T-cell lineage. Its clinical features overlap with those of several sinonasal mass lesions. While the histopathological features are well described, diagnosis is often difficult, owing to presence of extensive coagulative necrosis, so that repeated biopsies may sometimes be necessary for correct diagnosis. Literature on cytological findings of ENKTL is limited. Methods: Cytomorphological features of cases of histologically confirmed ENKTLhaving corresponding cytology samples were reviewed retrospectively, to identify distinctive features that could possibly suggest this entity. Results:Aspirates from five patients were studied: four from cervical nodes, one from cheek swelling and one from pleural fluid. Two aspirates were reported as positive for malignancy, two as atypical lymphoid proliferation and one was non-diagnostic. Pleural fluid was reported as malignant, favouring a diagnosis of carcinoma. On cytology, aspirates showed medium to large cells with folded, indented nuclei and abundant pale cytoplasm, some with tongue-like cytoplasmic protrusions. A distinctive feature was presence of large loose clusters of tumour cells with arborising capillaries running through them. Interestingly, necrosis was consistently absent.Subsequent biopsies from palate (three cases) and nasal masses (two cases) confirmed the diagnosis of ENKTL.Conclusions: Suspicion of ENKTL on cytology is crucial for timely diagnosis to avoid diagnostic delay, especially when only highly necrotic biopsy samples are available.Awareness of distinctive cytomorphological features is required to make fine needle aspiration an effective diagnostic tool for initial diagnosis and for evaluation of possible recurrences. K E Y W O R D Saspiration cytology, fluid cytology, haematolymphoid, NK/T-cell lymphoma, sinonasal

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Extranodal Natural Killer/T-Cell Lymphoma (ENKTL) Malignant Pleural Effusion: A Rare Finding in an Uncommon Disease

Cited by 2 publications

References 0 publications

Malignant effusions from extranodal NK/T‐cell lymphomas are frequently of anaplastic morphology with azurophilic granules and of T‐cell lineage

Malignant effusions from extranodal NK/T‐cell lymphomas are frequently of anaplastic morphology with azurophilic granules and of T‐cell lineage

Spectrum of cytomorphological features of extranodal NK/T‐cell lymphoma, nasal type

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