{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,23]],"date-time":"2026-02-23T10:14:04Z","timestamp":1771841644381,"version":"3.50.1"},"reference-count":24,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2026,2,23]],"date-time":"2026-02-23T00:00:00Z","timestamp":1771804800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dentistry Journal"],"abstract":"<jats:p>Background\/Objectives: Extranodal NK\/T-cell lymphoma, nasal type (ENKTCL-NT), is a rare and extremely aggressive subtype of non-Hodgkin lymphoma that most frequently involves the nasal cavity and upper aerodigestive tract. Primary isolated oral manifestation is exceptionally uncommon and may mimic odontogenic or infectious diseases, delaying diagnosis. We report a case of ENKTCL-NT presenting initially as a destructive oral lesion without sinonasal involvement at diagnosis. Methods: A 32-year-old man with progressive palatal ulceration underwent clinical and imaging assessment (panoramic radiography and staging ^18F-FDG PET\u2013CT) and repeated biopsies. Diagnosis was established using histopathology (H&amp;E), immunohistochemistry (T-cell markers and cytotoxic profile), EBV detection by EBER in situ hybridization, and T-cell receptor gamma (TCRG) gene rearrangement analysis. Results: The lesion presented as a hemorrhagic, ulcerative palatal destruction covered by pseudomembranous exudate and was complicated by fungal infection, periostitis, and severe dental inflammatory foci, contributing to diagnostic delay. Histopathological examination revealed extensive necrosis with a dense atypical lymphoid infiltrate; angiocentric and angiodestructive growth was identified in one biopsy specimen. Tumor cells expressed T-cell markers (CD2, CD3, CD5, CD7; heterogeneous) and cytotoxic markers (TIA-1) and showed CD30 and CD56 positivity, with EBV positivity confirmed by EBER in situ hybridization. Molecular analysis demonstrated monoclonal TCRG rearrangement, and Ki-67 indicated high proliferative activity. Initial PET\u2013CT demonstrated an intensely FDG-avid, locally invasive lesion without distant organ involvement. The patient was treated with L-asparaginase-based SMILE chemotherapy followed by radiotherapy (50 Gy), achieving marked initial clinical improvement and partial metabolic response; however, systemic relapse subsequently occurred with refractory disease despite salvage therapy and immunotherapy. Conclusions: This case highlights the substantial diagnostic challenge posed by isolated oral extranodal NK\/T-cell lymphoma, nasal type, which may closely mimic benign inflammatory or infectious conditions and lead to significant diagnostic delay. Persistent, progressive, or therapy-resistant oral ulcerations should prompt early consideration of hematologic malignancy. Timely biopsy with comprehensive immunophenotyping, EBV testing, and close multidisciplinary collaboration are essential for accurate diagnosis and may contribute to earlier diagnosis and improved patient outcomes in these rare and atypical presentations.<\/jats:p>","DOI":"10.3390\/dj14020129","type":"journal-article","created":{"date-parts":[[2026,2,23]],"date-time":"2026-02-23T08:58:21Z","timestamp":1771837101000},"page":"129","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Extranodal NK\/T-Cell Lymphoma, Nasal Type, Presenting as an Isolated Oral Manifestation"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-2680-991X","authenticated-orcid":false,"given":"Andrea","family":"Kanizsai","sequence":"first","affiliation":[{"name":"Maxillofacial Division, Oral and Maxillofacial Surgery, Department of Dentistry, Medical School, University of P\u00e9cs, R\u00e1k\u00f3czi Street 2, H-7623 P\u00e9cs, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u00c1gnes","family":"B\u00e1n","sequence":"additional","affiliation":[{"name":"Division of Periodontology, Oral and Maxillofacial Surgery, Department of Dentistry, Medical School, University of P\u00e9cs, T\u00fcz\u00e9r Street 1, H-7623 P\u00e9cs, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L\u00e1szl\u00f3","family":"Kereskai","sequence":"additional","affiliation":[{"name":"Clinical Center, Department of Pathology, Medical School, University of P\u00e9cs, Szigeti Street 12, H-7624 P\u00e9cs, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u00c1rp\u00e1d","family":"Szomor","sequence":"additional","affiliation":[{"name":"Janus Pannonius Clinical Block, Hematology Department, Clinical Center, University of P\u00e9cs, Ifj\u00fas\u00e1g Road 13, H-7624 P\u00e9cs, Hungary"},{"name":"Hematology Department, Szent Gy\u00f6rgy University Hospital, Sereg\u00e9lyesi Road 3, H-8000 Sz\u00e9kesfeh\u00e9rv\u00e1r, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,2,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"872","DOI":"10.1016\/j.resinv.2025.07.002","article-title":"Extranodal natural killer\/T-cell lymphoma with lung involvement in a young adult with suspected mosquito bite hypersensitivity: A case report","volume":"63","author":"Higa","year":"2025","journal-title":"Respir. Investig."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1635","DOI":"10.1556\/650.2017.30871","article-title":"Nasalis t\u00edpus\u00fa extranodalis natural killer T-sejtes lymphoma hazai el\u0151fordul\u00e1sa \u00e9s kezel\u00e9s\u00e9vel szerzett tapasztalatok","volume":"158","author":"Bakos","year":"2017","journal-title":"Orvosi Hetil."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"141","DOI":"10.11604\/pamj.2018.30.141.7721","article-title":"Lymphome T\/NK nasal: \u00c0 propos de deux cas rares","volume":"30","author":"Bouayad","year":"2018","journal-title":"Pan Afr. Med. J."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1720","DOI":"10.1038\/s41375-022-01620-2","article-title":"The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms","volume":"36","author":"Alaggio","year":"2022","journal-title":"Leukemia"},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Costa, R.d.O., Pereira, J., Lage, L.A.d.P.C., and Baiocchi, O.C.G. (2023). Extranodal NK-\/T-cell lymphoma, nasal type: What advances have been made in the last decade?. Front. Oncol., 13.","DOI":"10.3389\/fonc.2023.1175545"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"E39","DOI":"10.1002\/dc.25244","article-title":"A case of extranodal NK\/T-cell lymphoma, nasal type, diagnosed by scraping cytology of the maxillary gingiva","volume":"52","author":"Niwa","year":"2024","journal-title":"Diagn. Cytopathol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"79","DOI":"10.12659\/AJCR.882802","article-title":"Extranodal nasal\u2013type NK\/T-cell lymphoma of the palate and paranasal sinuses","volume":"13","author":"Nikitakis","year":"2012","journal-title":"Am. J. Case Rep."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1111\/jop.13205","article-title":"Mature T\/NK-Cell lymphomas of the oral and maxillofacial region: A multi-institutional collaborative study","volume":"50","author":"Abrantes","year":"2021","journal-title":"J. Oral Pathol. Med."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Yan, Z., Yao, S., Wang, Z., Zhou, W., Yao, Z., and Liu, Y. (2023). Treatment of extranodal NK\/T-cell lymphoma: From past to future. Front. Immunol., 14.","DOI":"10.3389\/fimmu.2023.1088685"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"e2015052","DOI":"10.4084\/mjhid.2015.052","article-title":"Nasal NK\/T- cell lymphoma. Comparative analysis of a Mexican population","volume":"7","year":"2015","journal-title":"Mediterr. J. Hematol. Infect. Dis."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"28","DOI":"10.14740\/wjon1351","article-title":"Epidemiology and Clinical Characteristics of Non-Hodgkin Lymphoma in Mexico","volume":"12","year":"2021","journal-title":"World J. Oncol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"e284","DOI":"10.1016\/S2352-3026(19)30283-2","article-title":"Survival outcomes of patients with extranodal natural-killer T-cell lymphoma: A prospective cohort study from the international T-cell Project","volume":"7","author":"Fox","year":"2020","journal-title":"Lancet Haematol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1001","DOI":"10.1016\/S0198-8859(01)00301-9","article-title":"Genetic link between Asians and native Americans: Evidence from HLA genes and haplotypes","volume":"62","author":"Tokunaga","year":"2001","journal-title":"Hum. Immunol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1002\/1096-8652(200102)66:2<145::AID-AJH1033>3.0.CO;2-V","article-title":"Familial nasal NK\/T-cell lymphoma and pesticide use","volume":"66","author":"Kojya","year":"2001","journal-title":"Am. J. Hematol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/j.envpol.2017.08.028","article-title":"The association between non-Hodgkin lymphoma and organophosphate pesticides exposure: A meta-analysis","volume":"231","author":"Hu","year":"2017","journal-title":"Environ. Pollut."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1136\/jcp.2008.062976","article-title":"Spontaneous regression of natural killer cell lymphoma","volume":"62","author":"Isobe","year":"2009","journal-title":"J. Clin. Pathol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1016\/j.humpath.2008.08.020","article-title":"Extranodal nasal-type natural killer\/T-cell lymphoma of the skin: A clinicopathologic study of 16 cases in China","volume":"40","author":"Yu","year":"2009","journal-title":"Hum. Pathol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1016\/j.clindermatol.2019.10.008","article-title":"Cutaneous leishmaniasis: A great imitator","volume":"38","author":"Gurel","year":"2020","journal-title":"Clin. Dermatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1016\/j.bjorl.2014.05.013","article-title":"Clinical and otorhinolaryngological aspects of extranodal NK\/T cell lymphoma, nasal type","volume":"80","author":"Miyake","year":"2014","journal-title":"Braz. J. Otorhinolaryngol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1097\/PAS.0b013e31824433d8","article-title":"Extranodal NK\/T-cell Lymphoma, Nasal Type, Includes Cases of Natural Killer Cell and \u03b1\u03b2, \u03b3\u03b4, and \u03b1\u03b2\/\u03b3\u03b4 T-cell Origin","volume":"36","author":"Pongpruttipan","year":"2012","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1016\/j.beha.2013.04.003","article-title":"International analysis of the frequency and outcomes of NK\/T-cell lymphomas","volume":"26","author":"William","year":"2013","journal-title":"Best Pract. Res. Clin. Haematol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1007\/s00430-018-0570-1","article-title":"Molecular mechanisms of EBV-driven cell cycle progression and oncogenesis","volume":"208","author":"Yin","year":"2019","journal-title":"Med. Microbiol. Immunol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1907059","DOI":"10.1080\/2162402X.2021.1907059","article-title":"VISTA and PD-L1 synergistically predict poor prognosis in patients with extranodal natural killer\/T-cell lymphoma","volume":"10","author":"He","year":"2021","journal-title":"Oncoimmunology"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1002\/hon.2944","article-title":"Review on natural killer\/T-cell lymphoma","volume":"41","author":"He","year":"2023","journal-title":"Hematol. Oncol."}],"container-title":["Dentistry Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2304-6767\/14\/2\/129\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,23]],"date-time":"2026-02-23T09:23:55Z","timestamp":1771838635000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2304-6767\/14\/2\/129"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,23]]},"references-count":24,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2026,2]]}},"alternative-id":["dj14020129"],"URL":"https:\/\/doi.org\/10.3390\/dj14020129","relation":{},"ISSN":["2304-6767"],"issn-type":[{"value":"2304-6767","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,23]]}}}