Is There a Role for Surgery in Treating Localized Esophageal Neuroendocrine Tumor?

Abstract

Primary esophageal neuroendocrine tumors (NET) are extremely rare, accounting for 0.5–5.9% of all esophageal cancers (which represents 0.04–1.4% of all gastrointestinal NETs). Unlike esophageal adenocarcinoma and squamous cell carcinoma, no standard guidelines are currently available for both staging and treating esophageal NET. However, in daily practice, esophageal NET is often treated similarly to small cell lung cancer due to the fact that they are systemic diseases sharing similar characteristics. As a result, non-surgical therapy has been recommended as the prior option for esophageal NET, and the role of surgery in treating esophageal NET remains poorly established. Recently, Erdem et al. explored the role of surgery in treating esophageal NET by including in their study a total of 250 patients with localized esophageal NET from the National Cancer Database. They found that patients with esophageal NET receiving surgical resection yielded a significantly higher overall survival (OS) rate than those treated with non-surgical therapy (2-year OS rate: 57.3% vs. 35.2%; p\\ 0.001), and the survival benefit of surgery still held in both the multivariable and propensity score-matched analyses. Therefore, their study suggested that surgery might improve the prognosis of patients with localized esophageal NET, and there was a role for surgery in treating localized esophageal NET. However, should we recommend surgery to all localized esophageal NET patients? In our previous study, we also investigated the role of surgery in treating localized esophageal NET. We included a total of 72 patients who were treated with esophagectomy for localized esophageal NET in our hospital and analyzed their prognosis. We found that the 3-year OS rate of patients with stage I/II disease was significantly higher than patients with stage III disease (45.0% vs. 16.1%). Compared with a median survival time of 8–16.7 months in patients treated with non-surgical therapy in previous studies, patients with stage III disease in our study yielded a similarly poor prognosis even after surgical resection (median OS time: 12.7 months). Therefore, patients with stage I/II disease seemed to benefit from surgery, while for patients with stage III disease, surgery may not improve their prognosis. Considering the finding from Erdem et al. showing that in stage I/II patients surgery still yielded a significantly better OS than non-surgical therapy, we believe that surgery does have a role in treating esophageal NET patients, but not for all localized esophageal NET patients. Similarly, a previous study also found that surgery could improve the survival of patients with stage I/II esophageal NET. Therefore, localized esophageal NET could be further subgrouped into ‘surgery response’ and ‘surgery non-response’ groups according to disease stage. We have therefore proposed an appropriate treatment algorithm for esophageal NET based on previous evidence. For patients with stage I/II disease, surgery plus chemoradiotherapy is suggested, while for patients with stage III disease or higher, non-surgical chemoradiotherapy might be the optimal treatment.