Laryngeal Neuroendocrine Carcinoma: A Population-Based Analysis of Incidence and Survival

Objective Laryngeal neuroendocrine carcinoma (LNEC) is a rare malignancy with various subtypes, each with different characteristics. Classification of these subtypes is used to delineate treatment and management, as most are clinically aggressive with poor prognosis. This study analyzes the characteristics and survival outcomes of LNEC using population-based data. Study Design Analysis of a population-based tumor registry. Setting Academic medical center. Subjects and Methods The Surveillance, Epidemiology, and End Results (SEER) database (1973-2011) was queried for LNEC cases. Data analyzed included patient demographics, incidence, treatment modality, and survival. Results In total, 257 LNEC cases were extracted from the SEER database. Sixty-three percent were male, and the mean age of diagnosis was 61.9 years. Most cases were located in the supraglottis (62.6%), were of the small cell carcinoma (52.9%) histologic subtype, and were grade IV (40.9%) and American Joint Committee on Cancer (AJCC) stage IV (59.4%). Surgery and radiotherapy were used as treatment modalities in 38.3% and 59.8% of patients, respectively. Overall 5-year disease-specific survival (DSS) for all LNEC was 30.2%, with lower grade, lower AJCC stage, and treatment with surgery having higher 5-year DSS. Conclusions LNEC often presents as an aggressive tumor at an advanced stage and has poor survival outcomes. Poor prognostic factors include high histologic grade, advanced stage disease, and not undergoing surgical resection. LNEC may be best treated depending on its histologic differentiation, with surgery being beneficial for early grade tumors while radiotherapy is inconclusive in its benefit for late-stage disease.