{"entity":{"id":"mucoepidermoid-carcinoma","kind":"cancer","name":"Mucoepidermoid carcinoma","aka":["MEC","Mucoepidermoid carcinoma of the salivary glands"],"tldr":"Mucoepidermoid carcinoma is the most common salivary gland cancer and, for most people, one of the most curable: low-grade tumours are removed surgically and rarely return, while high-grade tumours need radiotherapy after surgery and are treated like other aggressive head and neck cancers if they spread.","summary":"Mucoepidermoid carcinoma is made of mucous, intermediate and squamous-like (epidermoid) cells in varying proportions, and most tumours carry a CRTC1-MAML2 or CRTC3-MAML2 fusion that drives CREB-regulated genes and marks a better outlook. It arises in the parotid, the minor salivary glands of the palate and lips, and the bronchi, and it is the commonest salivary cancer in children and after radiotherapy or chemotherapy for a childhood cancer. Grade, assigned by histological systems such as the AFIP and Brandwein scores, is the strongest predictor of behaviour: low-grade tumours are indolent, high-grade tumours spread to nodes and distant sites.\n\nTreatment is surgical. Low-grade tumours are cured by complete excision with a margin (superficial or total parotidectomy preserving the facial nerve, or excision of the palatal lesion), and do not need radiotherapy when margins are clear. High-grade and intermediate-grade tumours, and any with positive margins, perineural invasion or nodal spread, are treated with neck dissection and postoperative radiotherapy, with cisplatin chemoradiation considered for extranodal extension or positive margins by analogy with squamous cell carcinoma.\n\nThere is no targeted therapy: the MAML2 fusion is not directly druggable, though it drives amphiregulin and EGFR signalling that has been explored preclinically. Recurrent or metastatic high-grade disease is treated with platinum-based chemotherapy, with pembrolizumab for the uncommon PD-L1-positive or mutation-rich tumour, and patients are offered the pan-salivary trials of new agents. Indolent low-grade metastases, which are rare, may be observed.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Mucoepidermoid_carcinoma","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Mucoepidermoid_carcinoma"}],"tags":["subtype-page","head-and-neck"],"related":[],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":["paper-bernier-eortc-22931-nejm-2004","paper-tonon-crtc1-maml2-nat-genet-2003","paper-laurie-licitra-salivary-systemic-jco-2006"],"journals":[],"dependsOn":[],"notes":[],"group":"head and neck","burden":"The commonest salivary gland cancer in both adults and children, most often in the parotid or the minor glands of the palate; low-grade tumours are cured by surgery in nearly every case, while high-grade tumours behave like other aggressive head and neck cancers.","subtypes":["Low-grade mucoepidermoid carcinoma (mostly cured by surgery)","Intermediate-grade mucoepidermoid carcinoma","High-grade mucoepidermoid carcinoma","CRTC1-MAML2 or CRTC3-MAML2 fusion-positive disease (the majority, better outlook)","Mucoepidermoid carcinoma of the palate and other minor salivary glands","Paediatric mucoepidermoid carcinoma, including after radiotherapy or chemotherapy"],"biomarkers":["MAML2 rearrangement by FISH (confirms the diagnosis)","Histological grade (AFIP or Brandwein system)","Margin status and perineural invasion","Nodal spread and extranodal extension","Ki-67"],"standardOfCare":[{"setting":"Low-grade, localised","approach":"Complete excision with a margin (parotidectomy preserving the facial nerve, or excision of the minor gland lesion); postoperative radiotherapy only for positive margins that cannot be re-excised.","refs":["imrt-igrt"],"guideline":{"nccn":"Category 2A","version":"NCCN Guidelines: Head and Neck Cancers","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1437"}},{"setting":"Intermediate- and high-grade or node-positive","approach":"Resection with neck dissection and postoperative radiotherapy; cisplatin chemoradiation considered for extranodal extension or positive margins.","refs":["imrt-igrt","cisplatin","chemoradiation"],"guideline":{"nccn":"Category 2A","version":"NCCN Guidelines: Head and Neck Cancers","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1437"}},{"setting":"Recurrent or metastatic","approach":"Platinum-based chemotherapy (carboplatin-paclitaxel or cisplatin-based); pembrolizumab for PD-L1-positive or mutation-rich tumours; observation for indolent low-grade metastases.","refs":["cisplatin","carboplatin","paclitaxel","pembrolizumab","pd-l1-testing"],"guideline":{"nccn":"Category 2A","version":"NCCN Guidelines: Head and Neck Cancers","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1437"}},{"setting":"Trials","approach":"Pan-salivary and pan-tumour trials of new agents that accept mucoepidermoid carcinoma.","refs":["gotistobart","nct05377996"]}],"stateOfArt":["Grade and margins, not molecular markers, still decide treatment.","The MAML2 fusion confirms the diagnosis and predicts a better course but has no drug.","Facial-nerve-sparing parotidectomy cures most patients with low-grade disease with little morbidity."],"history":[{"year":1945,"title":"Stewart, Foote and Becker name mucoepidermoid tumours of the salivary glands","refs":[]},{"year":2003,"title":"CRTC1-MAML2 fusion identified as the defining genetic event","refs":[]},{"year":2017,"title":"WHO classification confirms MAML2 fusion as diagnostic and prognostic","refs":[]}],"pipeline":["gotistobart","nct05377996","pembrolizumab"],"openProblems":["No targeted drug for the MAML2 fusion.","Grading systems disagree, so treatment intensity varies between centres.","High-grade disease has outcomes as poor as squamous cell carcinoma.","Second cancers in children treated for the disease."],"parent":"salivary-gland"},"route":"/cancers/mucoepidermoid-carcinoma/","neighbours":{"paper":[{"id":"paper-bernier-eortc-22931-nejm-2004","kind":"paper","name":"EORTC 22931: postoperative irradiation with or without concomitant cisplatin for locally advanced head and neck cancer","route":"/key-papers/paper-bernier-eortc-22931-nejm-2004/"},{"id":"paper-laurie-licitra-salivary-systemic-jco-2006","kind":"paper","name":"Systemic therapy in the palliative management of advanced salivary gland cancers (review)","route":"/key-papers/paper-laurie-licitra-salivary-systemic-jco-2006/"},{"id":"paper-tonon-crtc1-maml2-nat-genet-2003","kind":"paper","name":"The t(11;19) translocation in mucoepidermoid carcinoma creates a CRTC1-MAML2 fusion","route":"/key-papers/paper-tonon-crtc1-maml2-nat-genet-2003/"}],"drug":[{"id":"carboplatin","kind":"drug","name":"Carboplatin","route":"/drugs/carboplatin/"},{"id":"cisplatin","kind":"drug","name":"Cisplatin","route":"/drugs/cisplatin/"},{"id":"gotistobart","kind":"drug","name":"Gotistobart","route":"/drugs/gotistobart/"},{"id":"paclitaxel","kind":"drug","name":"Paclitaxel / nab-paclitaxel","route":"/drugs/paclitaxel/"},{"id":"pembrolizumab","kind":"drug","name":"Pembrolizumab","route":"/drugs/pembrolizumab/"}],"trial":[{"id":"nct05377996","kind":"trial","name":"A Study of Emiltatug Ledadotin (Emi-Le) in Participants With Solid Tumors","route":"/trials/nct05377996/"}],"technology":[{"id":"imrt-igrt","kind":"technology","name":"IMRT / IGRT (modern external beam)","route":"/technologies/imrt-igrt/"}],"term":[{"id":"chemoradiation","kind":"term","name":"Chemoradiation (chemoradiotherapy, CRT)","route":"/terms/chemoradiation/"},{"id":"extranodal-extension","kind":"term","name":"Extranodal extension (ENE)","route":"/terms/extranodal-extension/"},{"id":"pd-l1-testing","kind":"term","name":"PD-L1 expression testing (22C3, SP142, SP263)","route":"/terms/pd-l1-testing/"}],"cancer":[{"id":"salivary-gland","kind":"cancer","name":"Salivary gland cancers","route":"/cancers/salivary-gland/"}]}}