Carcinoids and High-Grade Neuroendocrine Carcinomas of the Ampulla of Vater: A Comparative Analysis of 139 Cases From the Surveillance, Epidemiology, and End Results Program—A Population Based Study

Abstract

Abstract Context.—Neuroendocrine tumors of the ampulla of Vater constitute a heterogeneous group of neoplasms clinically and morphologically. Because they are rare, little is known about their demographics and biologic behavior. Objective.—To analyze the demographics and the 5- and 10-year relative survival rates of 139 patients with carcinoid tumors and high-grade neuroendocrine carcinomas of the ampulla. Design.—Using data from National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) Program from 1973 to 2006, we analyzed the demographics, morphology, and survival of patients with carcinoids and neuroendocrine carcinomas of the ampulla. Results.—SEER recorded 6081 cases of malignant neoplasms of the ampulla, of which 82 were carcinoid tumors and 57 were high-grade neuroendocrine carcinomas. Of these 57, 42 were neuroendocrine carcinomas, not otherwise specified; 9 were small cell carcinomas; and 6 were large cell neuroendocrine carcinomas. The incidence was higher in men than in women. Patients with carcinoid tumors were younger (mean age, 61.6 years) than those with high-grade neuroendocrine carcinomas (mean age, 67.5 years). Carcinoid tumors were smaller than high-grade neuroendocrine carcinomas. The frequency of lymph node metastasis was 28.5% for carcinoid tumors and 62% for high-grade neuroendocrine carcinomas. The 5- and 10-year relative survival rates of patients with carcinoid tumors were 82% and 71%, respectively. The 5- and 10-year relative survival rate of patients with high-grade neuroendocrine carcinomas was 15.7%. Conclusions.—Carcinoids of the ampulla of Vater are relatively rare. Carcinoids and high-grade neuroendocrine carcinomas of the ampulla are biologically and clinically similar to these tumors arising in other sites. Carcinoids were smaller and metastasized less frequently than high-grade neuroendocrine carcinomas.