Abstract
BACKGROUND: Cases of undifferentiated cancers of the digestive system (UCDS) are occasionally encountered in clinical practice; however, no large-scale studies have described their characteristics. This study aimed to investigate the incidence, prognosis, and treatment strategies of UCDS. METHODS: We used the data from the Surveillance, Epidemiology, and End Results database to evaluate clinical characteristics in UCDS patients. A cohort of 1,372 patients was included to estimate UCDS incidence; another cohort of 1,816 patients was followed up until December 31, 2017 to evaluate treatment and prognosis. The incidence, trends, treatments, overall survival, and cause-specific survival were assessed. RESULTS: The estimated incidence of UCDS was 0.136 per 100,000 people-years [95% confidence interval (CI): 0.129-0.143] from 1975 to 2016 with respect to sex, age, year of diagnosis, race, region, site, income, and education. The age-adjusted rates of UCDS dropped from 0.567 per 100,000 people-years in 1978 to 0.031 per 100,000 people-years in 2016. The rates of surgery, beam radiation, and chemotherapy in patients with UCDS were 37.3%, 17.3%, and 30.7%, respectively. Overall, the 5-year cause-specific survival rate was 18%, with a median cause-specific survival of 4 months. Patient prognosis improved significantly over the decades; stage, site, year of diagnosis, surgery, beam radiation, and chemotherapy were independent prognostic factors for UCDS mortality. CONCLUSIONS: The incidence of UCDS has decreased in the United States over the past 40 years. Patients benefited from surgery, beam radiation, and chemotherapy. The survival of patients with UCDS has improved. Further research on developing decision-making recommendations for UCDS treatment is crucial.