Sinonasal neuroendocrine carcinoma: a population-based analysis of incidence and survival.

Abstract

Sinonasal neuroendocrine carcinoma (SNEC) is a rare, aggressive tumor usually associated with a poor prognosis. This study analyzes the clinicopathological characteristics and survival outcomes of SNEC using population-based data. The Surveillance, Epidemiology, and End Results (SEER) database (1973 to 2011) was queried for SNEC cases. Data analyzed included patient demographics, incidence, treatment modality, and survival. A total of 201 cases of SNEC were identified. Mean age at diagnosis was 55.8 ± 15.7 years. Overall 5-year disease-specific survival (DSS) rate for SNEC was 50.8%. Five-year survival analysis for SNEC by site revealed DSS of 80.7%, 59.2%, 34.5%, and 33.0% for the sphenoid sinus, nasal cavity, maxillary sinus, and ethmoid sinus, respectively (p = 0.0014). Cox proportional hazard analysis revealed greater hazard of death for the maxillary (hazard ratio [HR] 2.14; 95% confidence interval [CI], 1.21 to 3.71; p = 0.0094) and ethmoid sinuses (HR 1.83; 95% CI, 1.05 to 3.16; p = 0.0345) when compared to the nasal cavity. Advanced stage disease (stages III to IV, 5-year DSS 40.5%, p = 0.0008) was associated with poor survival outcomes. Survival was better among those treated with surgery (with [59.4%] or without [69.0%] radiotherapy) than those treated with primary radiotherapy alone (39.9%) (p < 0.0001). SNEC commonly presents at an advanced-stage with poor survival outcomes. Negative prognostic factors include primary tumor site and advanced stage disease. SNEC is a highly aggressive tumor necessitating surgery and/or surgery with adjuvant radiotherapy as the treatment of choice.