Signet ring cell carcinoma of the Ampulla of Vater: outcomes of patients in the United States

Abstract

Background: Signet ring cell carcinoma (SRCC) of the ampulla of Vater is poorly understood, with approximately 22 reported cases. Our study sought to create a comprehensive review of cases in the United States.

Methods: We used the Surveillance, Epidemiology, and End Results Program to collect all cases of ampullary adenocarcinoma diagnosed between 2010 and 2015.

Results: The age-adjusted incidence rate of SRCC of the ampulla of Vater was 1.2 cases per 10,000,000 persons per year, with 50% more cases in males than females. We identified 3448 cases of adenocarcinoma of the ampulla of Vater, 81 of which were SRCC (2.3%). SRCC tended to present a later stage than other ampullary cancers, with median survival times of 17 vs. 25 months, (p = 0.07). Survival was significantly worse for SRCC when accounting for other clinical features (HR 1.46, p = 0.01). Factors portending worse prognosis in SRCC of the ampulla of Vater were advanced age, late stage and lack of surgical intervention.

Conclusion: Our study represents the largest study of SRCC of the ampulla of Vater to date. SRCC has a poorer prognosis compared with other ampullary cancers. Optimal treatment regimen is the most important future area of study.