Esophageal adenocarcinoma in a 40-year-old man with cystic fibrosis: coincidence or not?
To report a case of esophageal cancer in an adult patient with cystic fibrosis (CF) and review the relationship between these 2 diseases. A 40-year-old man with CF presented with worsening epigastric pain, weight loss, and upper gastrointestinal (GI) bleeding. Endoscopy revealed innumerable masses in the distal esophagus. The workup revealed esophageal adenocarcinoma metastatic to the liver and the lungs. Abnormal mucous secretions in CF patients impair the innate GI mucosal barriers. The incidence of both gastroesophageal reflux disease and GI malignancies is higher in patients with CF. Patients with CF now survive long enough to potentially experience the consequences of long-term acid exposure, including esophagitis, Barrett esophagus, and esophageal cancer. Our case report adds to a small but growing body of evidence that CF is a significant risk factor for GI malignancies, including esophageal adenocarcinoma. Controlled studies are needed to determine whether a causal relationship truly exists.