{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T15:10:09Z","timestamp":1756307409146,"version":"3.44.0"},"reference-count":13,"publisher":"BMJ","issue":"1","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2025,1,3]]},"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:p>Salivary gland malignancies are rare, accounting for less than 5% of head and neck cancers. Mucoepidermoid carcinoma (MEC) is the most common salivary gland tumour, predominantly found in the parotid gland. However, it has rarely been reported in the tongue. Histologically, these tumours typically exhibit a combination of mucous, squamous and intermediate cell components. In the 2022 version of the WHO classification of salivary gland tumours, <jats:italic>MAML2<\/jats:italic> rearrangement is desirable but not essential for diagnosis. In rare cases, MEC may lack a squamous component, posing diagnostic challenges. In such cases, <jats:italic>MAML2<\/jats:italic> rearrangement is crucial for diagnosis. We present a unique case of a man in his 60s with a MEC arising in the tongue with predominantly clear cells and devoid of squamous components. This case emphasises the critical role of <jats:italic>MAML2<\/jats:italic> genetic testing in achieving an accurate diagnosis in rare locations with unusual morphology.<\/jats:p>","DOI":"10.1136\/bcr-2024-261310","type":"journal-article","created":{"date-parts":[[2025,1,21]],"date-time":"2025-01-21T23:45:37Z","timestamp":1737503137000},"page":"e261310","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Mucoepidermoid carcinoma lacking squamous component arising in rare location: utility of <i>MAML2<\/i> genetic testing for diagnosis"],"prefix":"10.1136","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-4065-8084","authenticated-orcid":false,"given":"Mckinzie","family":"Johnson","sequence":"first","affiliation":[{"name":"Department of Pathology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sanjana","family":"Mehrotra","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,1,21]]},"reference":[{"key":"2025082707343276000_18.1.e261310.1","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1007\/s10006-023-01163-5","article-title":"Head and neck mucoepidermoid carcinoma: does AFIP histological grading correlate with rate of nodal metastases?","volume":"28","author":"Selvarajah","year":"2024","journal-title":"Oral Maxillofac Surg"},{"key":"2025082707343276000_18.1.e261310.2","doi-asserted-by":"crossref","first-page":"1029","DOI":"10.5306\/wjco.v11.i12.1029","article-title":"Overall and cause-specific survival for mucoepidermoid carcinoma of the major salivary glands: Analysis of 2210 patients","volume":"11","author":"Taylor","year":"2020","journal-title":"World J Clin Oncol"},{"key":"2025082707343276000_18.1.e261310.3","doi-asserted-by":"crossref","unstructured":"Peraza A , G\u00f3mez R , Beltran J , et al . An update and review of the literature. J Stomatol Oral Maxillofac Surg 2020;Available from. doi:10.1016\/j.jormas.2020.06.003","DOI":"10.1016\/j.jormas.2020.06.003"},{"key":"2025082707343276000_18.1.e261310.4","doi-asserted-by":"crossref","DOI":"10.1177\/2473974X18791559","article-title":"Mucoepidermoid Carcinoma of the Tongue Base: A Case Report","volume":"2","author":"Zahran","year":"2018","journal-title":"OTO Open"},{"key":"2025082707343276000_18.1.e261310.5","unstructured":"Leivo I . Mucoepidermoid carcinoma. WHO Classification of Tumours Online. n.d. Available: https:\/\/tumourclassification.iarc.who.int\/chaptercontent\/52\/77"},{"key":"2025082707343276000_18.1.e261310.6","doi-asserted-by":"crossref","first-page":"4195","DOI":"10.1111\/cas.14632","article-title":"Prognostic impact of CRTC1\/3-MAML2 fusions in salivary gland mucoepidermoid carcinoma: A multiinstitutional retrospective study","volume":"111","author":"Okumura","year":"2020","journal-title":"Cancer Sci"},{"key":"2025082707343276000_18.1.e261310.7","first-page":"5","article-title":"Mucoepidermoid carcinoma: a clinico-pathological review of 75 cases","volume":"3","author":"Qureshi","year":"2012","journal-title":"Int J Oral Maxillofac Pathol"},{"key":"2025082707343276000_18.1.e261310.8","doi-asserted-by":"publisher","DOI":"10.1111\/jtm.12115"},{"key":"2025082707343276000_18.1.e261310.9","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000932"},{"key":"2025082707343276000_18.1.e261310.10","doi-asserted-by":"crossref","first-page":"141","DOI":"10.4103\/0973-029X.92994","article-title":"Clear cell variant of intraosseous mucoepidermoid carcinoma: Report of a rare entity","volume":"16","author":"Varma","year":"2012","journal-title":"J Oral Maxillofac Pathol"},{"key":"2025082707343276000_18.1.e261310.11","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1111\/his.14586","article-title":"Salivary mucoepidermoid carcinoma: histological variants, grading systems, CRTC1\/3-MAML2 fusions, and clinicopathological features","volume":"80","author":"Nakano","year":"2022","journal-title":"Histopathology"},{"key":"2025082707343276000_18.1.e261310.12","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1007\/s12105-013-0466-8","article-title":"Hyalinizing Clear Cell Carcinoma of Salivary Gland: A Review and Update","volume":"7","author":"Weinreb","year":"2013","journal-title":"Head and Neck Pathol"},{"key":"2025082707343276000_18.1.e261310.13","doi-asserted-by":"crossref","DOI":"10.1172\/jci.insight.139497","article-title":"The CRTC1-MAML2 fusion is the major oncogenic driver in mucoepidermoid carcinoma","volume":"6","author":"Chen","year":"2021","journal-title":"JCI Insight"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2024-261310","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T14:34:39Z","timestamp":1756305279000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2024-261310"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1]]},"references-count":13,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2025,1,21]]},"published-print":{"date-parts":[[2025,1]]}},"alternative-id":["10.1136\/bcr-2024-261310"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2024-261310","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2025,1]]}}}