Esophageal Cancer in a Young Patient With Celiac Disease: A Rare Association

Abstract

Introduction: Risk of malignancy including lymphoma and small intestinal cancer is increased in Celiac Disease (CD). Earlier studies from the United Kingdom have also suggested a link between celiac disease and esophageal carcinoma, although this has not been confirmed in North America. This association remains a rare occurrence and is almost never seen in younger patients. We report a case of celiac disease that developed esophageal carcinoma. Case summary: 35 years old lady developed chronic diarrhea five and a half years ago. Diarrhea was associated with abdominal pain and weight loss. She lost up to 10 kg weight. Initial laboratory investigations showed iron deficiency anemia, hypoalbuminemia, decreased vitamin D and calcium. The serology for CD was positive and she was placed on a gluten free diet to which she responded. Diarrhea and abdominal pain also improved. She remained symptoms free for almost 5 years. She then presented with new onset dysphagia initially for solids and then for liquids. Barium swallow showed a shelf like projection extending into the esophageal lumen suggestive of an esophageal web. There was narrowing of the distal esophagus but Barium flowed across this area with ease. She then underwent an upper endoscopy at which time scope could not be passed beyond the web. It was dilated using a 40 mm dilator and scope was then passed to about 25 cm from the incisors. A growth was seen protruding into the esophagus at about 25 cm that almost totally occluded the lumen. Multiple biopsies were taken. This extended to the GE junction and was seen involving the gastric fundus at the gastroesophageal junction. Multiple biopsies taken. Duodenum showed decrease number and height of the folds and the biopsy also was consistent with CD. Histopathology of esophageal growth showed squamous cell carcinoma while in stomach it showed moderate chronic non specific inflammation with focal surface ulceration. Discussion: Malignant disease is a serious concern in celiac disease. Diagnosed celiac disease has been traditionally linked with greatly increased risks of lymphoma and other malignancies. An increased risk of esophageal and pharyngeal carcinoma have been shown in some European studies. Patients of CD who are undiagnosed or do not adhere to strict gluten-free diet are prone to develop complications especially malignancy. However, these findings have not been confirmed in America. Risk of malignancy persists despite of gluten free diet.This case is an unusual case of a young patient with CD and Esophageal malignancy at a very young age. It highlights the need for aggressive follow-up along with screening of malignancies associated with CD.Figure 1Figure 2