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


CONCLUSIONS
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.
Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ.
Tapan D
TD
Patel
Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ.
Alejandro
A
Vazquez
Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ.
Pariket M
PM
Dubal
Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ.|Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Rutgers New Jersey Medical School, Newark, NJ.
Soly
S
Baredes
Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ.|Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Rutgers New Jersey Medical School, Newark, NJ.|Department of Neurological Surgery, Rutgers New Jersey Medical School, Newark, NJ.
James K
JK
Liu
Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ.|Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Rutgers New Jersey Medical School, Newark, NJ.|Department of Neurological Surgery, Rutgers New Jersey Medical School, Newark, NJ.
Jean Anderson
JA
Eloy
D000328:Adult; D000368:Aged; D018278:Carcinoma, Neuroendocrine / Q000401:mortality* / Q000473:pathology; D016208:Databases, Factual; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D010255:Paranasal Sinus Neoplasms / Q000401:mortality* / Q000473:pathology; D015996:Survival Rate
SEER; demographic; disease-specific survival; incidence; malignancy; nasal cancer; neuroendocrine carcinoma; sinonasal carcinoma; sinonasal malignancy; sinus cancer