Neuroendocrine neoplasms (NENs) of the esophagus are very uncommon with only a few studies published worldwide. Studies on clinical profile, management and outcomes are very uncommon. The aim was to study the clinicopathological characteristics and outcomes of neuroendocrine neoplasms of the esophagus.
We report the largest single institution retrospective review of 43 patients of esophageal NENs out of our registry of gastrointestinal neuroendocrine tumors treated between 2005 and 2014. Data on the incidence, tumor location, clinical symptoms, stage at presentation, grading, treatment protocol and treatment outcomes was collected and analyzed.
Among 124 NENs arising in different gastrointestinal sites, esophageal NENs were found in 43 cases. The mean patient age was 55.8 years. The male: female ratio was 1.5:1. 81.4 % of the tumors were located in the lower third of the esophagus and gastro-esophageal junction. Neuroendocrine carcinomas (NEC; G3) accounted for the vast majority of NENs (83.7%). 53.5 % patients were Stage IV and 32.5 % were Stage III at presentation. The combined median survival of Stages II and III patients was 18.25 months, with treatment. The median survival of treated patients with metastatic disease was 6.5 months.
Esophageal NENs most commonly were neuroendocrine carcinomas, presented in metastatic stage and were associated with poor prognosis. Grade 1 (G1) and Grade 2 (G2) had better outcome than NEC (G3). In non-metastatic disease, presence of lymph node metastasis and unresectable disease had poorer outcomes.
Clinical trial identification
All authors have declared no conflicts of interest.