Segmental xanthomatosis of the small intestine. A case report and review of the literature.

Intestinal xanthomatosis is a rare, nonneoplastic lesion that may involve the small bowel in a localized or generalized way. It most probably represents a nonspecific response to a previous injury. Most cases are not suspected clinically. They are diagnosed by endoscopy/biopsy, surgical specimen, or autopsy. It may be a cause of clinically significant obstruction and should be included in the differential diagnosis with other causes of bowel obstruction, particularly in patients with history of radiation therapy or chemotherapy. We report a case of segmental xanthomatosis involving 50 cm of the terminal ileum. The patient was a 22-year-old man who developed intestinal obstruction 16 years after radiation therapy for Ewing sarcoma of the right hip. A review of the English literature revealed that only rare cases have been previously reported.