Clinical Rhinology

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VOLUME 14 , ISSUE 1--3 ( January-December, 2023 ) > List of Articles

CASE REPORT

Natural Killer/T-cell Lymphoma of Nose—A Diagnostic Dilemma and Its Clinical Impact: A Case Report

Nitya Goddanti, Sudarshan Loka Reddy, Mounika Reddy Yalaka, Swetha Chevula, Trinath Yama

Keywords : Case report, Lymphoma, Nasal midline swelling, Sinonasal malignancy

Citation Information : Goddanti N, Reddy SL, Yalaka MR, Chevula S, Yama T. Natural Killer/T-cell Lymphoma of Nose—A Diagnostic Dilemma and Its Clinical Impact: A Case Report. Clin Rhinol An Int J 2023; 14 (1--3):25-28.

DOI: 10.5005/jp-journals-10013-1386

License: CC BY-NC 4.0

Published Online: 21-03-2024

Copyright Statement:  Copyright © 2023; The Author(s).


Abstract

Aim: To study the clinical course, investigatory findings, and the need for timely diagnosis of extranodal natural killer/T-cell lymphoma, nasal type (ENKTL-NT) and to understand the diagnostic difficulties and treatment options. Background: Extranodal natural killer/T-cell lymphoma, also known as “Stewart's Granuloma,” is a rare non-Hodgkin's lymphoma originating in the nasal cavity. It mostly arises from the malignant transformation of cytotoxic T cells. It is characterized by aggressive and rapidly progressing necrotic lesions involving the mid face, principally the nose and palate. It is a diagnosis made by the exclusion of other infective and granulomatous mid-facial lesions. Case description: A 35-year-old male presented with bilateral nasal obstruction for 20 days, left cheek swelling for 2 days, associated with progressive left-eye swelling and nasal regurgitation of fluids. Local examination showed extensive nasal crusting, and septal and palatal perforation. No clinical improvement was seen with empirical antibiotics and antifungals. Immunohistochemistry (IHC) was positive for cluster of differentiation (CD) 3 and 56, which led to a diagnosis of ENKTL-NT, and chemotherapy was started. However, he succumbed to coronavirus disease-2019 (COVID-19) disease during the course of treatment. Conclusion: Extranodal natural killer/T-cell lymphoma, nasal type is an aggressive form of non-Hodgkin's lymphoma with a clinical picture masquerading as acute invasive fungal sinusitis, mid-face granulomatous lesions, etc. This leads to delayed diagnosis, which in turn affects morbidity and mortality. Therefore, a timely and precise diagnosis with aggressive treatment is a must. Clinical significance: Extranodal natural killer/T-cell lymphoma, nasal type, is rare with rapid and aggressive local tissue necrosis and progression. Its refractory behavior to our initial treatment regimens raised suspicions. In the current challenging times, trying to decrease misdiagnosis is of paramount importance. Through this case report, we aim to alert fellow practitioners to keep this entity in the differential diagnosis of such an aggressive clinical picture with a short history.


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  1. Haverkos BM, Pan Z, Gru AA, et al. Extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT): An update on epidemiology, clinical presentation, and natural history in North American and European cases. Curr Hematol Malig Rep 2016;11(6):514–527. DOI: 10.1007/s11899-016-0355-9.
  2. Mehta V, Balachandran C, Bhat S, et al. Nasal NK/T-cell lymphoma presenting as a lethal midline granuloma. Indian J Dermatol Venereol Leprol 2008;74(2):145–147. DOI: 10.4103/0378-6323.39701.
  3. Patel V, Mahajan S, Kharkar V, et al. Nasal extranodal NK/T-cell lymphoma presenting as a perforating palatal ulcer: A diagnostic challenge. Indian J Dermatol Venereol Leprol 2006;72(3):218–221. DOI: 10.4103/0378-6323.25784.
  4. Aozasa K, Ohsawa M, Tajima K, et al. Nation-wide study of lethal mid-line granuloma in Japan: Frequencies of Wegener's granulomatosis, polymorphic reticulosis, malignant lymphoma and other related conditions. Int J Cancer 1989;44(1):63–66. DOI: 10.1002/ijc.2910440112.
  5. Swerdlow SH, Campo E, Harris N. et al. Classification of Tumours of Haematopoietic and Lymphoid Tissues Lyon, France: IARC Press; 2008.
  6. Kohrt H, Advani R. Extranodal natural killer/T-cell lymphoma: Current concepts in biology and treatment. Leuk Lymphoma 2009;50;(11):1773–1784. DOI: 10.3109/10428190903186502.
  7. Kato N, Yasukawa K, Onozuka T, et al. Nasal and nasal type T/NK cell lymphoma with cutaneous involvement. J Am Acad Dermatol 1999;40(5 Pt 2);850–856. DOI: 10.1053/jd.1999.v40.a94087.
  8. Reddy RR, Singh G, Prathima KJ, et al. CD3 positive extranodal T-cell lymphoma of nasal type with skin involvement. Indian J Dermatol Venereol Leprol 2006;72(3):215–217. DOI: 10.4103/0378-6323.25783.
  9. Zhong BN, Zhang XH, Li M, et al. Study of the pathology, immunophenotype, etiology and genetic markers of NK/T-cell lymphoma. Zhonghua Xue Ye Xue Za Zhi 2003;24(10):505–509. PMID: 14690577.
  10. Cristina OL, Cristian A, Mihail D. Lethal midline granuloma–Stewart nasal NK/T-cell lymphoma: Our experience. Glob J Oto 2018;14(4):555893. DOI: 10.19080/GJO.2018.14.555893.
  11. Batra P, Shah N, Mathur S. Midline lethal granuloma: A clinical enigma. Indian J Dent Res 2003;14(3):174–183. PMID: 15164661.
  12. Suzuki R. Pathogenesis and treatment of extranodal natural killer/T-cell lymphoma. Semin Hematol 2014;51(1):42–51. DOI: 10.1053/j.seminhematol.2013.11.007.
  13. Suzuki R, Suzumiya J, Yamaguchi M, et al. Prognostic factors for mature natural killer (NK) cell neoplasms: Aggressive NK cell leukemia and extranodal NK cell lymphoma, nasal type. Ann Oncol 2010;21:1032–1040. DOI: 10.1093/annonc/mdp418.
  14. Au W, Weisenburger DD, Intragumtornchai T, et al. Clinical differences between nasal and extranasal natural killer/T-cell lymphoma: A study of 136 cases from the International Peripheral T-Cell Lymphoma Project. Blood 2009;113(17):3931–3937. DOI: 10.1182/blood-2008-10-185256.
  15. Yan Z, Huang HQ, Wang XX, et al. A TNM staging system for nasal NK/T-cell lymphoma. PLoS One 2015;10(6):e0130984. DOI: 10.1371/journal.pone.0130984.
  16. Hartig G, Montone K, Wasik M, et al. Nasal T-cell lymphoma and lethal mid-line granuloma syndrome. Otolaryngol Head Neck Surg 1996;114(4):653–656. DOI: 10.1016/S0194-59989670264-4.
  17. Wang B, Li XQ, Ma X, et al. Immunohistochemical expression and clinical significance of P-glycoprotein in previously untreated extranodal NK/T-cell lymphoma, nasal type. Am J Hematol 2008;83:795–799. DOI: 10.1002/ajh.21256.
  18. Liu S, Zhou X, Song A, et al. Nasal-type extranodal natural killer/T-cell lymphoma presenting with a mass on the buttock: A case report. Medicine 2019;98(49);e18260. DOI: 10.1097/MD.0000000000018260.
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