An Unusual Presentation of Midline Lethal Granuloma.

Epstein-barr virus infections Extranodal NK-T-cell lymphoma Granuloma Lethal midline mass Lymphoma, non Hodgkin Metastasis

Journal

Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India
ISSN: 2231-3796
Titre abrégé: Indian J Otolaryngol Head Neck Surg
Pays: India
ID NLM: 9422551

Informations de publication

Date de publication:
Apr 2024
Historique:
received: 29 11 2023
accepted: 21 12 2023
pmc-release: 01 04 2025
medline: 3 4 2024
pubmed: 3 4 2024
entrez: 3 4 2024
Statut: ppublish

Résumé

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.

Identifiants

pubmed: 38566723
doi: 10.1007/s12070-023-04466-x
pii: 4466
pmc: PMC10982166
doi:

Types de publication

Journal Article

Langues

eng

Pagination

2137-2140

Informations de copyright

© 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.

Déclaration de conflit d'intérêts

Conflict of interestAll authors have no conflict of interest.

Auteurs

Ein Wan Chin (EW)

Department of Otorhinolaryngology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.

Jeyasakthy Saniasiaya (J)

Department of Otorhinolaryngology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.

Shahrul Hitam (S)

Department of Otorhinolaryngology, Hospital Ampang, Ampang, Selangor Malaysia.

Eyzawiah Hassan (E)

Department of Otorhinolaryngology, Hospital Ampang, Ampang, Selangor Malaysia.
Faculty of Medicine & Health Sciences, Universiti Sains Islam Malaysia, Negeri Sembilan, Malaysia.

Ngan Kah Wai (N)

Department of Pathology, Serdang Hospital, Serdang, Selangor Malaysia.

Classifications MeSH