Contemporary outcomes of esophageal and gastroesophageal junction neuroendocrine tumors

Adenocarcinoma and squamous cell esophageal cancers have been extensively studied in the literature. Esophageal neuroendocrine (NET)/carcinoid tumors are less commonly studied and have only been described in small series. The purpose of this study was to describe the demographics and natural history of esophageal NETs, as well as optimal treatments. We hypothesized that surgical resection would be the best treatment of esophageal NETs. The National Cancer Database was used to identify adult patients with esophageal or gastroesophageal junction (GEJ) cancer from 2004 to 2018. Patients were characterized as carcinoid/NET, adenocarcinoma, or squamous cell cancer. Clinical and demographic characteristics were compared between the histology groups. The primary outcome was overall survival, which was assessed by multivariable Cox analysis. Multivariable Cox analysis was also used to analyze factors associated with survival among NET patients who underwent surgery. Among 206,321 patients with esophageal cancer, 1,563 were NETs (<0.01%). Relative to the other two histologies, NETs were associated with younger age, female sex, and advanced clinical stage at diagnosis. Multivariate analysis suggested that NETs were less likely to be treated with surgical resection (OR 0.51, P < 0.001). Nonetheless, surgical resection was associated with improved survival (HR 0.64, P = 0.003). Among patients with NETs who received surgery, neoadjuvant therapy was associated with improved overall survival (HR 0.38, P = 0.013). NET of the esophagus presents with more advanced disease than other common histologies. Among patients with nonmetastatic cancer, surgical resection appears to be the best treatment. Neoadjuvant systemic therapy may offer survival benefit, but future studies are necessary.