Multifocal adenocarcinoma of the proximal small intestine in a patient with celiac sprue

Adenocarcinoma of the small intestine is a known complication of celiac sprue. We report a 63-year-old man with a history of celiac disease who developed anemia, guaiac-positive stools, and jaundice. Five discrete adenocarcinomas of the proximal small intestine were identified over a 9 year period. Endoscopic retrograde cholangiopancreatographic (ERCP) and radiographic evaluation of the small bowel were helpful in diagnosing adenocarcinoma involving the ampulla of Vater and jejunum. This case demonstrates for the first time the multifocal, malignant potential of sprue. It also illustrates the importance of an aggressive, systematic work-up of patients whose course has changed despite dietary restriction.