Abstract
BACKGROUND: Epithelial-myoepithelial carcinoma (EMCa) is a low-grade malignant tumor that occurs primarily in the salivary glands. Its occurrence in the nasopharynx is exceedingly rare. Due to its scarcity, there is currently a lack of standardized treatment protocols, and robust prognostic data remain limited. METHODS: We report a case series of two patients with histopathologically confirmed nasopharyngeal EMCa, supplemented by a systematic literature review to summarize its clinicopathological characteristics, treatment modalities, and prognostic factors. RESULTS: The two cases exhibited strikingly divergent outcomes. The first patient (Case 1) underwent surgical resection followed by adjuvant radiotherapy, achieving a complete clinical response and remaining disease-free at 31 months. In contrast, the second patient (Case 2) presented a markedly more aggressive profile, characterized by younger age, neurological symptoms, and an atypically high (18)F-FDG uptake on PET/CT, a notable deviation from the typically non-significant avidity reported for EMCa. Despite aggressive multimodal therapy, the disease progressed rapidly with extensive metastases. Subsequent institution of a combined chemotherapy and immunotherapy regimen successfully achieved disease stabilization. CONCLUSION: Our cases illustrate the heterogeneous clinical behavior of nasopharyngeal EMCa, ranging from indolent disease to aggressive progression. Our observations suggest that high (18)F-FDG uptake may serve as a critical biomarker for an aggressive phenotype. Given this variability, individualized treatment strategies may be warranted, and further studies are needed to better define optimal management for this rare tumor.