Background and Objectives: Current guidelines of the American Joint Commission on Cancer (AJCC) for rectal neuroendocrine tumors (NETs) classify tumor nodal status as N0/N1. This staging does not take into consideration the number of positive lymph nodes. The goal of this study is to determine how the number of positive lymph nodes affects the prognosis for patients with rectal NETs. Methods: The National Cancer Database was used to identify patients with rectal NETs who underwent rectal resection. Nearest-neighborhood grouping was used to classify patients by survival to create a new nodal staging system. Results: There were 687 patients with rectal NETs. There were distinct 5-year survival estimates for patients with N0 [81.8% (95%CI:77.1%-85.6%)], N1 (1-4 positive lymph nodes) [57.8% (95% confidence interval (CI: 51.2%-63.9%)] and N2 (≥5 positive lymph nodes) [32.6% (95%CI:25.1%-40.3%)] patients, P < 0.0001. Distinct 5-year survival estimates using the new nodal staging system was apparent for patients in the external validation set. After adjusting for predictors of survival in multivariable analysis, the new nodal stage remained an independent predictor of overall survival. Conclusions: The number of positive locoregional lymph nodes is an independent prognostic factor in rectal NETs. The next AJCC edition should consider classifying patients with rectal NETs as N0, N1, and N2 to provide better estimates of survival for patients. K E Y W O R D S AJCC staging, carcinoid tumor, lymph nodes, rectal cancer, rectal neuroendocrine tumor 1 | INTRODUCTION Gastroenteropancreatic neuroendocrine tumors (NETs) have varied behaviors and prognoses depending on their location in the gastrointestinal tract. The rectum is one of the most common sites of gastrointestinal NETs with an age-adjusted incidence of approximately 1 per 100,000. 1,2 Furthermore, the incidence of rectal NETs has increased tenfold over the past 30 years. 3 Tumor size, depth of invasion, and lymph node status are important prognostic factors. 4-6 The five-year survival after diagnosis with rectal NETs is typically higher than other NET sites and exceeds 88%. 4 This is likely due to tumors being localized at the time of diagnosis. 2 Once rectal NETs metastasize, however, five-year survivaldrops to 36%-73% in the presence of regional lymph node metastasis and to 15%-30% for distant metastasis. 2,7,8 In 2010, the American Joint Commission on Cancer (AJCC) published its first TNM staging classification for rectal NETs; locoregional lymph node metastases were characterized as present or absent (N1/N0) and continue to be characterized in this system in the 2017 AJCC 8th edition. 9 This staging system matches the classification developed by the European Neuroendocrine Tumor Society (ENETS) and North American Neuroendocrine J Surg Oncol. 2019;119:156-162. wileyonlinelibrary.com/journal/jso 156 |