An Unusual Presentation of Midline Lethal Granuloma
© Association of Otolaryngologists of India 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law..
Extranodal natural killer T-cell lymphoma, nasal type (ENKTCL), is a rare form of non-Hodgkin lymphoma that is strongly related to Epstein-Barr Virus (EBV) infection and commonly presents as "midline lethal granuloma." Herein, we report a middle-aged lady who presented with a two-week history of fever, sore throat and constitutional symptoms. Intraoral examination revealed a lacerated soft palate with an ulcerated uvula. A diagnosis of ENKTCL was confirmed through deep biopsies under general anaesthesia supplemented with a positive serum EBV genome. Unfortunately, she succumbed due to disease progression with left frontal brain metastasis with concurrent pulmonary tuberculosis before treatment was completed. The recommended treatment is multimodality with L-asparaginase-containing regimes chemotherapy in an advanced stage, relapsed, or refractory ENKTCL for better outcomes. The quantification of circulating plasma EBV deoxyribonucleic acid (DNA) is helpful as the baseline of tumour load and a biomarker for monitoring treatment response and prognostication. We advocate repeated and deeper core tissue biopsies.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:76 |
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| Enthalten in: |
Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India - 76(2024), 2 vom: 03. Apr., Seite 2137-2140 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Chin, Ein Wan [VerfasserIn] |
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| Links: |
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| Themen: |
Epstein-barr virus infections |
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| Anmerkungen: |
Date Revised 04.04.2024 published: Print-Electronic Citation Status PubMed-not-MEDLINE |
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| doi: |
10.1007/s12070-023-04466-x |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
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| 520 | |a Extranodal natural killer T-cell lymphoma, nasal type (ENKTCL), is a rare form of non-Hodgkin lymphoma that is strongly related to Epstein-Barr Virus (EBV) infection and commonly presents as "midline lethal granuloma." Herein, we report a middle-aged lady who presented with a two-week history of fever, sore throat and constitutional symptoms. Intraoral examination revealed a lacerated soft palate with an ulcerated uvula. A diagnosis of ENKTCL was confirmed through deep biopsies under general anaesthesia supplemented with a positive serum EBV genome. Unfortunately, she succumbed due to disease progression with left frontal brain metastasis with concurrent pulmonary tuberculosis before treatment was completed. The recommended treatment is multimodality with L-asparaginase-containing regimes chemotherapy in an advanced stage, relapsed, or refractory ENKTCL for better outcomes. The quantification of circulating plasma EBV deoxyribonucleic acid (DNA) is helpful as the baseline of tumour load and a biomarker for monitoring treatment response and prognostication. We advocate repeated and deeper core tissue biopsies | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Epstein-barr virus infections | |
| 650 | 4 | |a Extranodal NK-T-cell lymphoma | |
| 650 | 4 | |a Granuloma | |
| 650 | 4 | |a Lethal midline mass | |
| 650 | 4 | |a Lymphoma, non Hodgkin | |
| 650 | 4 | |a Metastasis | |
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| 700 | 1 | |a Hitam, Shahrul |e verfasserin |4 aut | |
| 700 | 1 | |a Hassan, Eyzawiah |e verfasserin |4 aut | |
| 700 | 1 | |a Kah Wai, Ngan |e verfasserin |4 aut | |
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