Cohort study reveals histological grade and primary site dictate survival in mucoepidermoid carcinoma, highlighting marked prognostic heterogeneity across disease stages.
Background The purpose of this study was to characterize the clinicopathologic features, treatment patterns, long-term outcomes, and prognostic factors of mucoepidermoid carcinoma (MEC) in a real-world cohort. Materials and methods We retrospectively analyzed 283 patients with MEC treated between 2005 and 2025. Treatment included surgery, radiotherapy, and chemotherapy according to institutional protocols. Disease-free survival (DFS) and overall survival (OS) were estimated using the Kaplan–Meier method. Independent prognostic factors were evaluated using Cox proportional hazards models. Results With a median follow-up of 53 months (range 1-246), histological grading was available for 202 patients: 35.6% low-grade, 46.0% intermediate-grade, and 18.3% high-grade. The parotid gland (42.8%) and lung (19.4%) were the most common primary sites. Among 118 patients tested for cAMP response element–binding protein-regulated transcription coactivator 1/3–mastermind-like protein 2 (CRTC1/3–MAML2) fusion, 95 (80.5%) were fusion-positive, and fusion status was significantly associated with histological grade. Five-year DFS/OS rates were 100%/100% for low-grade, 88.2%/93.5% for intermediate-grade, and 70.7%/79.8% for high-grade tumors. High-grade tumors had significantly poorer survival than low- or intermediate-grade tumors, whereas low- and intermediate-grade tumors did not differ significantly, including after propensity score matching. Perineural invasion independently predicted DFS, whereas age, N classification, and primary site independently predicted OS. Conclusions MEC showed marked prognostic heterogeneity by histological grade and primary site. Intermediate-grade MEC showed relatively favorable outcomes, but matched analyses were limited by small sample size and few events. CRTC1/3–MAML2 fusion was associated with histological grade, whereas its prognostic value remains exploratory.
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