{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,18]],"date-time":"2026-04-18T19:05:54Z","timestamp":1776539154537,"version":"3.51.2"},"reference-count":21,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,4,30]],"date-time":"2021-04-30T00:00:00Z","timestamp":1619740800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Surg."],"abstract":"<jats:p><jats:bold>Background:<\/jats:bold> In the head and neck region the uvula is a rare site for extranodal lymphomas to develop. In this national study, we present six cases and provide an overview of the current literature, characterizing the clinical and histopathological features of lymphomas involving this location.<\/jats:p><jats:p><jats:bold>Materials and Methods:<\/jats:bold> Clinical information was obtained retrospectively from patient records in a nationwide Danish study covering from 1980 through 2019. In order to validate the diagnoses, uvular tissue specimens were examined histologically and immunohistochemically and if relevant for subtyping, cytogenetic rearrangements were investigated.<\/jats:p><jats:p><jats:bold>Results:<\/jats:bold> We present six cases of lymphomas involving the uvula, of which four of the cases were diagnosed with a B-cell lymphoma (two diffuse large B-cell lymphomas, one extranodal marginal zone B-cell lymphoma and one Mantle cell lymphoma), while two were diagnosed with a T-cell lymphoma (one peripheral T-cell lymphoma and one natural killer\/T-cell lymphoma). Presenting symptoms included swelling, pain and ulceration of the uvula. Treatment was comprised of radiotherapy and\/or chemotherapy, with T-cell lymphomas showing a poorer outcome than B-cell lymphomas.<\/jats:p><jats:p><jats:bold>Conclusion:<\/jats:bold> Lymphoma of the uvula is rare, with few case reports being reported in the literature. The most frequent histological subtypes reported are extranodal marginal zone B-cell lymphoma and peripheral T-cell lymphoma. When encountering a swollen, painful and\/or ulcerated uvula, the clinician should always consider malignancy as a possible cause. Lymphoma of the uvula is a possible diagnosis and if this is the case, there is a high risk of disseminated disease at the time of diagnosis.<\/jats:p>","DOI":"10.3389\/fsurg.2021.675279","type":"journal-article","created":{"date-parts":[[2021,4,30]],"date-time":"2021-04-30T04:28:36Z","timestamp":1619756916000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":4,"title":["Lymphoma of the Uvula: Clinical, Morphological, Histopathological, and Genetic Characterization. A Nationwide Danish Study From 1980 to 2019"],"prefix":"10.3389","volume":"8","author":[{"given":"Lars","family":"Iversen","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrick Rene Gerhard","family":"Eriksen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simon","family":"Andreasen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erik","family":"Clasen-Linde","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Preben","family":"Hom\u00f8e","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Irene","family":"Wessel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian","family":"von Buchwald","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Steffen","family":"Heegaard","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2021,4,30]]},"reference":[{"key":"B1","volume-title":"WHO Classification of Tumours of Haematopoetic and Lymphoid Tissues","author":"Swerdlow","year":"2008"},{"key":"B2","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1016\/S1052-5149(03)00039-X","article-title":"Hodgkin and non-Hodgkin lymphoma of the head and neck: clinical, pathologic, and imaging evaluation","volume":"13","author":"Weber","year":"2003","journal-title":"Neuroimaging Clin N Am"},{"key":"B3","doi-asserted-by":"publisher","first-page":"748","DOI":"10.1002\/hed.21022","article-title":"National cancer database report on cancer of the head and neck: 10-year update","volume":"31","author":"Cooper","year":"2009","journal-title":"Head Neck"},{"key":"B4","doi-asserted-by":"publisher","first-page":"649","DOI":"10.1007\/s00428-019-02543-7","article-title":"Lymphomas of the head and neck region: an update","volume":"474","author":"Cabe\u00e7adas","year":"2019","journal-title":"Virchows Arch"},{"key":"B5","doi-asserted-by":"publisher","first-page":"706","DOI":"10.1111\/apm.12407","article-title":"Activation of the interleukin-6\/Janus kinase\/STAT3 pathway in pleomorphic adenoma of the parotid gland","volume":"123","author":"Andreasen","year":"2015","journal-title":"Apmis"},{"key":"B6","doi-asserted-by":"publisher","first-page":"461","DOI":"10.3109\/10428190109058006","article-title":"MALT lymphoma originating in breast and uvula","volume":"41","author":"Okabe","year":"2001","journal-title":"Leuk Lymphoma"},{"key":"B7","doi-asserted-by":"publisher","first-page":"E22","DOI":"10.1177\/014556131209101218","article-title":"Low-grade B-cell lymphoma presenting as a uvular mass","volume":"91","author":"Walker","year":"2012","journal-title":"Ear Nose Throat J"},{"key":"B8","doi-asserted-by":"publisher","first-page":"1","DOI":"10.15761\/OHNS.1000139","article-title":"Primary malignant lymphoma of the uvula","volume":"2","author":"Kusunoki","year":"2017","journal-title":"Otolaryngol Head Neck Surg"},{"key":"B9","doi-asserted-by":"publisher","first-page":"358","DOI":"10.4103\/0019-5049.135094","article-title":"Right molar approach for uvulectomy of secondary non-hodgkins lymphoma of uvula","volume":"58","author":"Singh","year":"2014","journal-title":"Indian J Anaesth"},{"key":"B10","doi-asserted-by":"publisher","first-page":"608","DOI":"10.1093\/rheumatology\/kes372","article-title":"T-cell lymphoma manifesting as a uvular mass","volume":"52","author":"Lai","year":"2013","journal-title":"Rheumatol."},{"key":"B11","doi-asserted-by":"publisher","first-page":"32","DOI":"10.30699\/acadpub.mci.3.1.32","article-title":"Peripheral T-cell lymphoma of the uvula: a rare entity","volume":"3","author":"Binesh","year":"2019","journal-title":"Multidiscip Cancer Investig"},{"key":"B12","doi-asserted-by":"publisher","first-page":"101","DOI":"10.22038\/ijorl.2017.8269","article-title":"Extranodal NK\/T cell lymphoma with destruction of the uvulae: a case report","volume":"29","author":"Azad","year":"2017","journal-title":"Iran J Otorhinolaryngol"},{"key":"B13","doi-asserted-by":"publisher","first-page":"299","DOI":"10.1080\/000155500750012234","article-title":"Oral involvement in mycocis fungoides: report of two cases and a literature review","volume":"80","author":"Gomez-De La Fuente","year":"2000","journal-title":"Acta Derm Venereol"},{"key":"B14","doi-asserted-by":"publisher","first-page":"2873","DOI":"10.1007\/s00405-019-05544-z","article-title":"Oral lesion as the primary diagnosis of non-Hodgkin's lymphoma: a 20-year experience from an oral pathology service and review of the literature","volume":"276","author":"Kusuke","year":"2019","journal-title":"Eur Arch Oto-Rhino-Laryngology"},{"key":"B15","doi-asserted-by":"publisher","first-page":"519","DOI":"10.1067\/moe.2001.116062","article-title":"Characteristics of oral and paraoral malignant lymphoma: a population-based review of 361 cases","volume":"92","author":"Epstein","year":"2001","journal-title":"Oral Surg Oral Med Oral Pathol Oral Radiol Endod"},{"key":"B16","doi-asserted-by":"publisher","first-page":"263","DOI":"10.4236\/jct.2012.34037","article-title":"Oral squamous cell carcinoma: epidemiology, clinical presentation and treatment","volume":"3","author":"Feller","year":"2012","journal-title":"J Cancer Ther"},{"key":"B17","doi-asserted-by":"publisher","first-page":"3102","DOI":"10.1182\/blood-2007-05-075176","article-title":"Lymphoma dissemination: the other face of lymphocyte homing","volume":"110","author":"Pals","year":"2007","journal-title":"Blood"},{"key":"B18","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1016\/j.oraloncology.2017.08.018","article-title":"Oropharyngeal lymphoma: a US population based analysis","volume":"73","author":"Rayess","year":"2017","journal-title":"Oral Oncol"},{"key":"B19","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1016\/j.ijrobp.2015.01.009","article-title":"Modern radiation therapy for extranodal lymphomas: field and dose guidelines from the international lymphoma radiation oncology group","volume":"92","author":"Yahalom","year":"2015","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"B20","doi-asserted-by":"publisher","first-page":"578","DOI":"10.1182\/asheducation-2017.1.578","article-title":"Secondary CNS relapse in diffuse large B-cell lymphoma: defining high-risk patients and optimization of prophylaxis strategies","volume":"2017","author":"Savage","year":"2017","journal-title":"Hematology"},{"key":"B21","doi-asserted-by":"publisher","first-page":"1647","DOI":"10.1007\/s00277-019-03689-9","article-title":"Improved prognosis of extranodal NK\/T cell lymphoma, nasal type of nasal origin but not extranasal origin","volume":"98","author":"Yamaguchi","year":"2019","journal-title":"Ann Hematol"}],"container-title":["Frontiers in Surgery"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fsurg.2021.675279\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,4,30]],"date-time":"2021-04-30T04:28:40Z","timestamp":1619756920000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fsurg.2021.675279\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,4,30]]},"references-count":21,"alternative-id":["10.3389\/fsurg.2021.675279"],"URL":"https:\/\/doi.org\/10.3389\/fsurg.2021.675279","relation":{},"ISSN":["2296-875X"],"issn-type":[{"value":"2296-875X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,4,30]]},"article-number":"675279"}}