Late diagnosis: a case of rapidly progressive extranodal NK/T cell lymphoma, nasal type

Mori, Hiroyuki; Ebisawa, Kei; Nishimura, Mitsushige; Kanazawa, Kenji

doi:10.1136/bcr-2017-221019

Cited by 9 publications

(9 citation statements)

References 10 publications

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“…Obtaining tissue samples useful for diagnosis may not be possible in superficial punch biopsies, given the secondary rhinosinusitis usually found in nasal neoplasms 11. Conversely, surgery is rottenly necessary to perform deep tissue biopsy 12…”

Section: Discussionmentioning

confidence: 99%

Extranodal natural killer/T cell lymphoma, nasal type: a diagnostic challenge

et al. 2019

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Extranodal natural killer/T cell lymphoma, nasal type (ENKL) is a rare and aggressive tumour that can, clinically and histologically, mimic infectious and inflammatory conditions, presenting a diagnostic challenge. The authors report the case of a 69-year-old Portuguese woman previously misdiagnosed with chronic recurrent sinusitis. Despite maximal medical and surgical treatments, the disease was refractory and progressed. The patient had undergone multiple biopsies when the histopathological diagnosis of ENKL was made, 5 months after the initial complaints. Multiagent chemotherapy was offered, but during the first cycle, the patient developed severe infection and pancytopenia, which culminated in her death. This case highlights the need to consider a neoplastic cause when faced with aggressive sinonasal disease not responsive to maximal treatment and the difficulties in establishing the diagnosis of ENKL, with multiples biopsies of deep-tissue usually being required.

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Section: Discussionmentioning

confidence: 99%

Extranodal natural killer/T cell lymphoma, nasal type: a diagnostic challenge

et al. 2019

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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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Late diagnosis: a case of rapidly progressive extranodal NK/T cell lymphoma, nasal type

Cited by 9 publications

References 10 publications

Extranodal natural killer/T cell lymphoma, nasal type: a diagnostic challenge

Extranodal natural killer/T cell lymphoma, nasal type: a diagnostic challenge

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

Primary pulmonary extranodal natural killer/T-cell lymphoma of nasal type presenting as pneumonia in the right lower lobe: A case report

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