17 Cholangiocarcinoma: Incidence, Prevalence and Descriptive Analysis in the VA Caribbean Healthcare System in Puerto Rico

Abstract

INTRODUCTION: Cholangiocarcinoma (CCA) is a rare cancer that arises from the epithelial cells of the bile ducts. Although uncommon, it is highly lethal, since usually it is advanced at the time of presentation. According to the Global Burden of Disease Study there were 139,500 deaths from biliary tract cancers in 2013, which is a 22% increase from the estimated 115,400 deaths in 1990. The incidence and prevalence of CCA at the VA Caribbean Healthcare System (VACHS) in San Juan, Puerto Rico, has not been statistically determined. The aim of this study is to determine the incidence and prevalence of CCA and to provide a descriptive analysis of the veteran population at the VACHS. METHODS: Veteran male subjects ≥21 years old with a diagnosis of CCA as documented in ICD-9/ICD-10 were included in this study from 2001–2017. The data was collected from the electronic medical record at the VACHS. RESULTS: A total of fifty-seven patients were diagnosed with CCA by the ICD 9-10 criteria. Fifty-four confirmed by biopsy. The mean age of the subjects was 75 years old. The overall estimate prevalence of CCA is 88/100,000 population. According to the data collected, 63%, 30%, 5% and 2% patients with CCA served in the Korean, Vietnam, WWII and Persian Gulf wars, respectively. The most common comorbidities in the biopsy-proven subjects were diabetes mellitus type II (50%), cirrhosis (26.4%), cholelithiasis (21.8%) and obesity (10.5%). Among the patients who had underlying cirrhosis, 29% had HCV. A total of 29 patients (54%) had a positive history of tobacco use and 15% of ethanolism. The most common clinical presentations identified were painless jaundice (44.4%), weight loss (29.6%), RUQ pain (27.8%) and incidental finding on imaging (18.5%). Most of the cases (85%) presented with a cholestatic pattern on routine laboratories. A total of 59.6% of the CCAs were intrahepatic and 26.9% were located distally. At the time of diagnosis, more than half (51.2%) had distant metastasis. CONCLUSION: This clinical study, although limited by a small population size and the absence of a control group for comparison, describes the most common clinical and social characteristics related to CCA and its overall prevalence and incidence per year at the VACHS from 2001–2017. Although, the overall estimate prevalence of CCA in our population is low, the rarity of this tumor, along with its asymptomatic presentation and poor prognosis, supports the claim that further research is needed to achieve early detection and better outcomes.