Tapan D Patel1, Alejandro Vazquez1, Pariket M Dubal1, Soly Baredes1,2, James K Liu1,2,3, Jean Anderson Eloy1,2,3. 1. Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ. 2. Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Rutgers New Jersey Medical School, Newark, NJ. 3. Department of Neurological Surgery, Rutgers New Jersey Medical School, Newark, NJ.
Abstract
BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.
BACKGROUND: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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.
Authors: Lester D R Thompson; Vickie Y Jo; Abbas Agaimy; Antonio Llombart-Bosch; Gema Nieto Morales; Isidro Machado; Uta Flucke; Paul E Wakely; Markku Miettinen; Justin A Bishop Journal: Head Neck Pathol Date: 2017-09-05