BACKGROUND: Nasopharyngeal neuroendocrine carcinoma (NP-NEC) is a rare malignancy, and its clinical features, management, and prognosis remain poorly characterized. OBJECTIVES: To describe the clinicopathologic characteristics, treatment patterns, incidence, and survival of NP-NEC in a population-based cohort. METHODS: Patients diagnosed with NP-NEC between 2000 and 2022 were identified from the Surveillance, Epidemiology, and End Results Program using ICD-O-3 topography. Demographic, tumor, and treatment data were extracted. Overall survival (OS) and disease-specific survival (DSS) were estimated using Kaplan-Meier methods and compared using log-rank tests. RESULTS: The cohort comprised 96 patients, with a mean age of 56.98 years and a male predominance (68.8%). Small cell carcinoma was the predominant histologic subtype, accounting for 50% of cases. Most tumors were high-grade and advanced stage, and one-fifth of patients underwent surgery. Radiotherapy was delivered in 78.1%. Median OS and DSS were 21 and 23 months, with 5-year OS and DSS of 32.3% and 39.1%. Histologic subtype and AJCC stage were associated with both OS and DSS, and treatment modality was associated with DSS. CONCLUSIONS: NP-NEC is uncommon and carries a poor prognosis. Early-stage disease and carcinoid histology appear associated with favorable survival. Stage, histologic subtype, and definitive local therapy may be key determinants of outcome.
Lü et al. (Tue,) studied this question.