Extranodal NK/T Cell Lymphoma with Destruction of the Uvulae: A Case Report

Document Type : Case Report


1 Allergy Research Center, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

2 Oral and Maxillofacial Diseases Research Center, Faculty of Dentistry, Mashhad University of Medical Science, Mashhad, Iran.

3 Department of Oral and Maxillofacial Medicine, Faculty of Dentistry, Mashhad University of Medical Science, Mashhad, Iran.

4 Department of Hematology-Oncology, Mashhad University of Medical Science, Mashhad, Iran.


Extranodal Natural Killer (NK)/T-cell lymphoma (NKTCL) nasal type is a rare but well-known disease with poor prognosis. NKTCL is more prevalent in Asia and comprises about 7-10% of all non-Hodgkin lymphoma cases in this region. The characteristic clinical pattern of NKTCL is the destruction of the midline structures of the mid-face.
Case Report:
The present study examines a case of NKTCL in a 23-year-old man with a destructive ulcer of the palate and uvulae. Based on immunohistochemical results, after three months of delay, the definitive diagnosis was revealed to be Extranodal NK/T cell lymphoma. Following the third cycle of chemotherapy, the patient died due to sepsis and infection.
It is very common to misdiagnose NKTCL with other clinical conditions such as necrotizing stomatitis, deep fungal ulcers, Wegener’s Granulomatosis disease, etc. Delay in diagnosis can worsen the course of the disease and its prognosis.


Main Subjects

1. Neville BW, Damm DD, Allen CM, Chi AC. Oral and maxillofacial pathology. 4th ed. Riverport Lane St. Louis, Missouri: Elsevier. 2016.
2. Mestiri S, Zeglaoui I, Sriha B, Belcadhi M, Bouzouita K, Korbi S. [Extra-nodal T lymphomas of the nasal cavities and sinuses]. Annales d'oto-laryngologie et de chirurgie cervico faciale : bulletin de la Societe d'oto-laryngologie des hopitaux de Paris. 2008 Sep;125(4):188-92.
3. Tababi S, Kharrat S, Sellami M, Mamy J, Zainine R, Beltaief N, et al. Extranodal NK/T-cell lymphoma, nasal type: report of 15 cases. European annals of otorhinolaryngology, head and neck diseases. 2012 Jun;129(3):141-7.
4. Hmidi M, Kettani M, Elboukhari A, Touiheme N, Messary A. Sinonasal NK/T-cell lymphoma. European annals of otorhinolaryngology, head and neck diseases. 2013 Jun;130(3):145-7.
5. Zheng Y, Jia J, Li W, Wang J, Tian Q, Li Z, et al. Extranodal natural killer/T-cell lymphoma, nasal type, involving the skin, misdiagnosed as nasosinusitis and a fungal infection: A case report and literature review. Oncology letters. 2014 .Nov;8(5):2253-62.
6. Lee H, Kim SH, Lee SW, Zheng Z, Bang D, Kim DY. A Case of Extranodal Natural Killer/T-cell Lymphoma Mimicking Refractory Behçet’s Disease. 2015. Apr;95(4):491-2.
7. Au W-y, Weisenburger DD, Intragumtornchai T, Nakamura S, Kim W-S, Sng I, 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):3931-7.
8. Yoo JH, Kim SY, Jung KB, Lee JJ, Lee SJ. Intraocular involvement of a nasal natural killer T-cell lymphoma: a case report. Korean journal of ophthalmology: KJO. 2012; Feb;26(1):54-7.
9. Bhatt VR, Koirala B, Terjanian T. Extranodal natural killer/T cell lymphoma, nasal type presenting as a palatal perforation and naso-oral fistula. BMJ case reports. 2011 Mar 1:pii: bcr1120103511.
10. Moradi S, Chavoshzadeh Z, Izadyar M, Mahjoub F, Rezaei N. Angiocentric Nasal T-Cell Lymphoma in a Patient with Idiopathic CD4+ Lymphocytopenia. Iranian Journal of Allergy, Asthma and Immunology. 2009;8(4):215-8.
11. Andreoli TE, Benjamin I, Griggs RC, Wing EJ, Fitz JG. Andreoli and Carpenter's Cecil essentials of medicine: Elsevier Health Sciences; 2010.
12. Glick M. Burket's Oral Medicine:12th ed.PMPH-USA,LTD  shelton,  CT.2015.
13. Tlholoe MM, Kotu M, Khammissa RA, Bida M, Lemmer J, Feller L. Extranodal natural killer/T-cell lymphoma, nasal type: 'midline lethal granuloma.' A case report. Head Face Med. 2013;9:4.
14. Wood PB, Parikh SR, Krause JR. Extranodal NK/T-cell lymphoma, nasal type. Proceedings (Baylor University Medical Center). 2011 Jul;24(3):251-4.
15. Kidwai SM, Parasher AK, Lin FY. An unusual presentation of NK/T-cell lymphoma, nasal-type in the United States. American journal of otolaryngology. 2015 Jan-Feb;36(1):80-3.
16. Gu Y, Wu B, Zhang Y, Guo Y, Peng W. MRI appearances of stage IE/IIE extranodal NK/T-cell lymphoma, nasal type, in the upper aerodigestive tract. European review for medical and pharmacological sciences. 2014;18(3):404-12.
17. Bi X-W, Li Y-X, Fang H, Jin J, Wang W-H, Wang S-L, et al. High-Dose and Extended-Field Intensity Modulated Radiation Therapy for Early-Stage NK/T-Cell Lymphoma of Waldeyer's Ring: Dosimetric Analysis and Clinical Outcome. International Journal of Radiation Oncology* Biology* Physics. 2013;87(5):1086-93.
18. Chauchet A, Michallet AS, Berger F, Bedgedjian I, Deconinck E, Sebban C, et al. Complete remission after first-line radio-chemotherapy as predictor of survival in extranodal NK/T cell lymphoma. Journal of hematology & oncology. 2012;5:27.
19. MacDonald SL, Mulroy L, Wilke DR, Burrell S. PET/CT aids the staging of and radiotherapy planning for early-stage extranodal natural killer/T-cell lymphoma, nasal type: a case series. Radiat Oncol. 2011;6(2):182.
20. Meng W, Zhou Y, Zhang H, Jiang L, Wang Z, Li X, et al. Nasal-type NK/T-cell lymphoma with palatal ulcer as the earliest clinical manifestation: a case report with literature review. Pathology oncology research : POR. 2010 Mar;16(1):133-7.
21. Nikolaos N, Grigorios P, Konstantinos K, Savvas T, Vassiliki Z, Alexandra S, et al. Extranodal nasal-type NK/T-cell lymphoma of the palate and paranasal sinuses. The American journal of case reports. 2012;13:79-85.
22. Patel V, Mahajan S, Kharkar V, Khopkar U. Nasal extranodal NK/T-cell lymphoma presenting as a perforating palatal ulcer: a diagnostic challenge. Indian journal of dermatology, venereology and leprology. 2006 May-Jun; 72(3): 218-21.
23. Tsang WM, Tong AC, Lam KY, Tideman H. Nasal T/NK cell lymphoma: report of 3 cases involving the palate. Journal of oral and maxillofacial surgery :official journal of the American Association of Oral and Maxillofacial Surgeons. 2000 Nov;58(11):1323-7.
24. Al-Hakeem DA, Fedele S, Carlos R, Porter S. Extranodal NK/T-cell lymphoma, nasal type. Oral oncology. 2007 Jan;43(1):4-14.
25. Li S, Feng X, Li T, Zhang S, Zuo Z, Lin P, et al. Extranodal NK/T-cell lymphoma, nasal type: a report of 73 cases at MD Anderson Cancer Center. The American journal of surgical pathology. 2013 Jan;37(1):14-23.
26. Ferrazzo KL, Schneider PP, Shinohara EH. An unusual case of adenoid cystic carcinoma with hard palate perforation. Minerva stomatologica. 2011 Jan-Feb;60(1-2):83-6.
27. Hertel M, Matter D, Schmidt-Westhausen AM, Bornstein MM. Oral syphilis: a series of 5 cases. Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons. 2014 Feb;72(2):338-45.
28. Leuci S, Martina S, Adamo D, Ruoppo E, Santarelli A, Sorrentino R, et al. Oral Syphilis: a retrospective analysis of 12 cases and a review of the literature. Oral diseases. 2013 Nov;19(8):738-46.
29. Sarojini P, Basheer A. Perforation of the hard palate due to tuberculosis. Indian Journal of Dermatology, Venereology, and Leprology. 1978;44(2):114.
30. Trimarchi M, Bertazzoni G, Bussi M. Cocaine induced midline destructive lesions. Rhinology. 2014 Jun;52(2):104-11.
31. Trimarchi M, Gregorini G, Facchetti F, Morassi ML, Manfredini C, Maroldi R, et al. Cocaine-induced midline destructive lesions: clinical, radiographic, histopathologic, and serologic features and their differentiation from Wegener granulomatosis. Medicine. 2001 Nov;80(6):391-404.
32. Winters TS, Yaibuathes S, Khan R, Bizzarro RJ, Skiba JF, Clemente EA. Cocaine-induced palatal perforation: A case report. Journal of the New Jersey Dental Association. 2014 Spring; 85(2):19-20.
33. Ray P, Halder A, Chowdhury J, Roy AK. Primary Tuberculosis in Soft Palate: Case Report of a Rare Entity. Indian journal of dermatology. 2014 Jul;59(4):423.
34. Kosalka J, Bazan-Socha S, Zugaj A, Ignacak M, Zuk J, Sokolowska B, et al. Granulomatosis with polyangiitis (Wegener's granulomatosis) with hard palate and bronchial perforations treated with rituximab - a case report. Pneumonologia i alergologia polska. 2014;82(5):454-7.
35. Aries PM, Ullrich S, Gross WL. A case of destructive Wegener's granulomatosis complicated by cytomegalovirus infection. Nature clinical practice Rheumatology. 2006 Sep;2(9):511-5.
36. Ortu E, Pietropaoli D, Baldi M, Marzo G, Giannoni M, Monaco A. Polyarteritis nodosa involving the hard palate: a case report. Journal of medical case reports. 2013;7:79.
37. Seishima M, Yuge M, Kosugi H, Nagasaka T. Extranodal NK/T-cell lymphoma, nasal type, possibly arising from chronic Epstein-Barr virus infection. Acta dermato-venereologica. 2010;90(1):102-3.
38. Vasile D, Vladareanu AM, Bumbea H. Peripheral T and NK cell non-hodgkin lymphoma a challenge for diagnosis. Maedica. 2014 Mar;9(1):104-8.
39. Asano N, Kato S, Nakamura S. Epstein-Barr virus-associated natural killer/T-cell lymphomas. Best practice & research Clinical haematology. 2013 Mar;26(1):15-21.
40. Borisch B, Hennig I, Laeng RH, Waelti ER, Kraft R, Laissue J. Association of the subtype 2 of the Epstein-Barr virus with T-cell non-Hodgkin's lymphoma of the midline granuloma type. Blood. 1993 Aug 1;82(3):858-64.
 41. Aozasa K, Zaki MA. Epidemiology and pathogenesis of nasal NK/T-cell lymphoma: a mini-review. TheScientificWorldJournal. 2011; 11:422-8.
42. Balachandran C, Sabita L, Kanthraj GR. Perforation of hard palate in lues maligna associated with HIV infection. Genitourinary medicine. 1997 Jun;73(3):225.
43. Ashraf MJ, Makarempour A, Monabati A, Azarpira N, Khademi B, Hakimzadeh A, et al. Comparison between presence of epstein barr virus in nodal and extra nodal diffuse large B cell lymphoma of head and neck, an Iranian experience. Iranian Red Crescent medical journal. 2012 Dec;14(12):764-70.
44. Robinson CF, Dement JM, Ness GO, Waxweiler RJ. Mortality patterns of rock and slag mineral wool production workers: an epidemiological and environmental study. British journal of industrial medicine. 1982 Feb;39(1):45-53.
45. Cancer IAfRo, Humans IWGotEoCRt. IARC monographs on the evaluation of carcinogenic risks to humans: IARC; 2004.
46. Costa R, Orriols R. Man-made mineral fibers and the respiratory tract. Archivos de Bronconeumología (English Edition). 2012;48(12):460-8.
47. Suzuki R, Takeuchi K, Ohshima K, Nakamura S. Extranodal NK/T-cell lymphoma: diagnosis and treatment cues. Hematological oncology. 2008 Jun;26(2):66-72.