Abstract
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.
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs12070-023-04466-x/MediaObjects/12070_2023_4466_Fig1_HTML.png)
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs12070-023-04466-x/MediaObjects/12070_2023_4466_Fig2_HTML.png)
Similar content being viewed by others
References
Chaudhary RK, Bhatt VR, Vose JM (2015) Management of extranodal natural killer/t-cell Lymphoma, nasal type. Clin Lymphoma Myeloma Leuk 15(5):245–252
Mei M, Wang Y, Zhang M (2019) Causes of mortality in cases with extra nodal natural killer/T-cell Lymphoma, nasal type: a cohort study. PLoS ONE 14(4):e0214860
Asano N, Kato S, Nakamura S (2013) Epstein-Barr virus-associated natural killer/T-cell Lymphomas. Best Pract Res Clin Haematol 26(1):15–21
Salah HB, Fourati N, Sellami N, Ghorbel L, Ayedi L et al (2015) Treatment and outcomes of Centrofacial NK/T cell Lymphoma nasal type: a report of 8 cases and literature review. Ann Clin Pathol 3(1):1043
Tse E, Kwong YL (2017) The diagnosis and management of NK/T-cell Lymphomas. J Hematol Oncol 10(1):85
Bhatt VR, Koirala B, Terjanian T (2011) Extranodal natural killer/T cell Lymphoma, nasal type presenting as a palatal perforation and naso-oral fistula. BMJ Case Rep 2011:bcr1120103511
Mohd Ramli SS, Husain S, Wong YP (2021) Extranodal NK/T cell Lymphoma, nasal type: a rare diagnosis with common nasal presentation. BMJ Case Rep 14(6):e236436
Yamaguchi M, Kwong YL, Kim WS, Maeda Y, Hashimoto C, Suh C, Izutsu K, Ishida F, Isobe Y, Sueoka E, Suzumiya J, Kodama T, Kimura H, Hyo R, Nakamura S, Oshimi K, Suzuki R (2011) Phase II study of SMILE chemotherapy for newly diagnosed stage IV, relapsed, or refractory extranodal natural killer (NK)/T-cell Lymphoma, nasal type: the NK-Cell Tumor Study Group study. J Clin Oncol 29:4410–4416
Funding
None.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
All authors have no conflict of interest.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
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.
About this article
Cite this article
Chin, E.W., Saniasiaya, J., Hitam, S. et al. An Unusual Presentation of Midline Lethal Granuloma. Indian J Otolaryngol Head Neck Surg 76, 2137–2140 (2024). https://doi.org/10.1007/s12070-023-04466-x
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12070-023-04466-x