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.
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.
Treatment 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.
There 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.
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.
Site decides cause and behaviour: HPV drives oropharyngeal cancer, EBV drives nasopharyngeal cancer, tobacco drives oral and laryngeal cancer; all drain into the neck node levels that surgeons and radiotherapists map.
Same organ: Acinic cell carcinoma of the salivary glands, Carcinoma ex pleomorphic adenoma, Multiple endocrine neoplasia type 1 (MEN1), Multiple endocrine neoplasia type 2 (MEN2A and MEN2B), Hyperparathyroidism-jaw tumour syndrome (CDC73-related parathyroid carcinoma), Oropharyngeal cancer (tonsil and base of tongue), Laryngeal and hypopharyngeal cancer, Oral cavity cancer (mouth and tongue), Head and neck squamous cell carcinoma, Nasopharyngeal carcinoma, Salivary gland cancers, Papillary thyroid cancer, Follicular thyroid cancer, Medullary thyroid cancer, Anaplastic thyroid cancer, Thyroid cancer, Nasal cavity and paranasal sinus cancers (including esthesioneuroblastoma), NUT carcinoma (midline carcinoma with NUTM1 rearrangement), Parathyroid carcinoma, Multiple endocrine neoplasia syndromes (MEN1, MEN2, MEN4), HPV-positive oropharyngeal cancer, HPV-negative head and neck squamous cell carcinoma (including HPV-negative oropharyngeal cancer), Recurrent or metastatic head and neck squamous cell carcinoma, Hypopharyngeal cancer, Adenoid cystic carcinoma, Salivary duct carcinoma, Oral tongue and floor of mouth cancer, Buccal mucosa and gingivobuccal cancer (oral cancer in India), Lip cancer, Locoregionally advanced nasopharyngeal carcinoma (stage III to IVA), Recurrent and metastatic nasopharyngeal carcinoma, Esthesioneuroblastoma (olfactory neuroblastoma), Sinonasal undifferentiated carcinoma (SNUC) and SWI/SNF-deficient sinonasal carcinoma
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.
Resection with neck dissection and postoperative radiotherapy; cisplatin chemoradiation considered for extranodal extension or positive margins.
Platinum-based chemotherapy (carboplatin-paclitaxel or cisplatin-based); pembrolizumab for PD-L1-positive or mutation-rich tumours; observation for indolent low-grade metastases.
Pan-salivary and pan-tumour trials of new agents that accept mucoepidermoid carcinoma.
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The observation-first approach for indolent metastases and the move to biomarker-directed therapy (HER2, androgen receptor, NTRK) on the salivary cancer pages follow this review's conclusions.
Cisplatin chemoradiation after surgery is standard for extranodal extension or involved margins; radiotherapy alone suffices for other adverse features.
CRTC1-MAML2 fusion testing supports the diagnosis of mucoepidermoid carcinoma, particularly in difficult cases, and fusion-positive tumours tend to be lower grade with a better outlook.
Query for this cancer: (TITLE:"Mucoepidermoid carcinoma" OR ABSTRACT:"Mucoepidermoid carcinoma" OR TITLE:"MEC" OR ABSTRACT:"MEC" OR TITLE:"Mucoepidermoid carcinoma of the salivary glands" OR ABSTRACT:"Mucoepidermoid carcinoma of the salivary glands") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Mucoepidermoid carcinoma, not a curated reading list.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
No pharmacokinetic interactions expected (antibody). See the irAE guide for toxicity management.
Dose by Calvert formula using GFR (see the calculators).
Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
During chemotherapy a temperature over 37.5 C or below 36 C, shivering, or feeling unwell even with a normal temperature means ringing the hospital's 24-hour line straight away; breathing very fast, confusion, mottled skin or no urine in a day means 999.
See all on the product pages:CarboplatinCisplatinPaclitaxel / nab-paclitaxelPembrolizumab·Printable cards in the navigator
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